Questions About Cancer? 1-800-4-CANCER

Adult Non-Hodgkin Lymphoma Treatment (PDQ®)

Health Professional Version
Last Modified: 04/11/2014

Aggressive NHL

Diffuse Large B-cell Lymphoma
        Prognosis
        CNS prophylaxis
Primary Mediastinal Large B-cell Lymphoma
Follicular Large Cell Lymphoma
        Prognosis
        Therapeutic approaches
Anaplastic Large Cell Lymphoma
Extranodal NK-/T-cell Lymphoma
Lymphomatoid Granulomatosis
Angioimmunoblastic T-cell Lymphoma
Peripheral T-cell Lymphoma
        Prognosis
        Therapeutic approaches
Enteropathy-type Intestinal T-cell Lymphoma
Intravascular Large B-cell Lymphoma (Intravascular Lymphomatosis)
Burkitt Lymphoma/Diffuse Small Noncleaved-cell Lymphoma
        Therapeutic approaches
Lymphoblastic Lymphoma
Adult T-cell Leukemia/Lymphoma
Mantle Cell Lymphoma
        Therapeutic approaches
Polymorphic Posttransplantation Lymphoproliferative Disorder (PTLD)
        Prognosis
        Therapeutic options
True Histiocytic Lymphoma
        Therapeutic options
Primary Effusion Lymphoma
        Prognosis
        Therapeutic approaches

Aggressive non-Hodgkin lymphoma (NHL) includes the following subtypes:

Diffuse Large B-cell Lymphoma

Diffuse large B-cell lymphoma (DLBCL) is the most common of the NHLs and comprises 30% of newly diagnosed cases.[1] Most patients present with rapidly enlarging masses, often with both local and systemic symptoms (designated B symptoms with fever, recurrent night sweats, or weight loss). (Refer to the PDQ summary on Hot Flashes and Night Sweats and the PDQ summary on Nutrition in Cancer Care for more information on weight loss.)

Some cases of large B-cell lymphoma have a prominent background of reactive T cells and often of histiocytes, so-called T-cell/histiocyte-rich large B-cell lymphoma. This subtype of large cell lymphoma has frequent liver, spleen, and bone marrow involvement; however, the outcome is equivalent to that of similarly staged patients with DLBCL.[2-4] Some patients with DLCBL at diagnosis have a concomitant indolent small B-cell component; while overall survival (OS) appears similar after multidrug chemotherapy, there is a higher risk of indolent relapse.[5]

Prognosis

The vast majority of patients with localized disease are curable with combined–modality therapy or combination chemotherapy alone.[6] For patients with advanced-stage disease, 50% of presenting patients are cured with doxorubicin-based combination chemotherapy and rituximab.[7-9]

An International Prognostic Index (IPI) for aggressive NHL (diffuse large cell lymphoma) identifies five significant risk factors prognostic of OS:[10]

  1. Age (≤60 years vs. >60 years).
  2. Serum lactate dehydrogenase (LDH) (normal vs. elevated).
  3. Performance status (0 or 1 vs. 2–4).
  4. Stage (stage I or stage II vs. stage III or stage IV).
  5. Extranodal site involvement (0 or 1 vs. 2–4).

Patients with two or more risk factors have a less than 50% chance of relapse-free survival and OS at 5 years. This study also identifies patients at high risk of relapse based on specific sites of involvement, including bone marrow, central nervous system (CNS), liver, lung, and spleen. Age-adjusted and stage-adjusted modifications of this IPI are used for younger patients with localized disease.[11] The bcl-2 gene and rearrangement of the myc gene or dual overexpression of the myc gene, or both, confer a particularly poor prognosis.[12-15] Patients at high risk of relapse may be considered for clinical trials.[16] Molecular profiles of gene expression using DNA microarrays may help to stratify patients in the future for therapies directed at specific targets and to better predict survival after standard chemotherapy.[17-21]

CNS prophylaxis

CNS prophylaxis (usually with four to six injections of methotrexate intrathecally) is recommended for patients with paranasal sinus or testicular involvement. Some clinicians are employing high-dose intravenous methotrexate (usually four doses) as an alternative to intrathecal therapy because drug delivery is improved and patient morbidity is decreased.[22] CNS prophylaxis for bone marrow involvement is controversial; some investigators recommend it, others do not.[23,24] A retrospective analysis of 605 patients with diffuse large cell lymphoma who did not receive prophylactic intrathecal therapy identified an elevated serum LDH and more than one extranodal site as independent risk factors for CNS recurrence. Patients with both risk factors have a 17% probability of CNS recurrence at 1 year after diagnosis (95% confidence interval [CI], 7%–28%) versus 2.8% (95% CI, 2.7%–2.9%) for the remaining patients.[25][Level of evidence: 3iiiDiii] The addition of rituximab to CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)-based regimens has significantly reduced the risk of CNS relapse in retrospective analyses.[26,27]

Primary Mediastinal Large B-cell Lymphoma

Primary mediastinal (thymic) large B-cell lymphoma is a subset of diffuse large cell lymphoma characterized by significant fibrosis on histology.[28-34] Patients are usually female and young (median age of 30–40 years). Patients present with a locally invasive anterior mediastinal mass that may cause respiratory symptoms or superior vena cava syndrome.

Prognosis and therapy is the same as for other comparably staged patients with DLCBL. Uncontrolled, phase II studies employing dose-adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) plus rituximab or R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) show high cure rates while avoiding any mediastinal radiation.[35-38][Level of evidence: 3iiiA] These results suggest that patients who receive R-CHOP-based regimens may avoid the serious long-term complications of radiation therapy when given with chemotherapy. Posttreatment fluorine-18-fluorodeoxyglucose–positron-emission tomography–computed tomography (FDG-PET-CT) scans appear unreliable with many false positives.[35] The only randomized trial showing an OS advantage for combined modality therapy was retracted. (Refer to the Superior Vena Cava Syndrome section in the PDQ summary on Cardiopulmonary Syndromes for more information.)

Follicular Large Cell Lymphoma

Prognosis

The natural history of follicular large cell lymphoma remains controversial.[39] While there is agreement about the significant number of long-term disease-free survivors with early-stage disease, the curability of patients with advanced disease (stage III or stage IV) remains uncertain. Some groups report a continuous relapse rate similar to the other follicular lymphomas (a pattern of indolent lymphoma).[40] Other investigators report a plateau in freedom-from-progression at levels expected for an aggressive lymphoma (40% at 10 years).[41,42] This discrepancy may be caused by variations in histologic classification between institutions and the rarity of patients with follicular large cell lymphoma. A retrospective review of 252 patients, all treated with anthracycline-containing combination chemotherapy, showed that patients with more than 50% diffuse components on biopsy had a worse OS than other patients with follicular large cell lymphoma.[43]

Therapeutic approaches

Treatment of these patients is more similar to treatment of aggressive NHL than it is to the treatment of indolent NHL. In support of this approach, treatment with high-dose chemotherapy and autologous hematopoietic peripheral stem cell transplantation (SCT) shows the same curative potential in patients with follicular large cell lymphoma who relapse as it does in patients with diffuse large cell lymphoma who relapse.[44][Level of evidence: 3iiiA]

Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphomas (ALCL) may be confused with carcinomas and are associated with the Ki-1 (CD30) antigen. These lymphomas are usually of T-cell origin, often present with extranodal disease, and are found especially in the skin.

The translocation of chromosomes 2 and 5 creates a unique fusion protein with a nucleophosmin-ALK.[45]

Patients whose lymphomas express ALK (immunohistochemistry) are usually younger and may have systemic symptoms, extranodal disease, and advanced-stage disease; however, they have a more favorable survival rate than that of ALK-negative patients.[46,47]

Patients with anaplastic lymphoma kinase (ALK)-positive ALCL are generally treated the same as patients with diffuse large cell lymphomas using the CHOP regimen and have a prognosis that is as good as comparably staged patients.[47] For patients with relapsed disease, anecdotal responses have been reported for brentuximab vedotin (anti-tubulin agent attached to a CD30-specific monoclonal antibody) [48,49], romidepsin [50], and pralatrexate.[51][Level of evidence: 3iiiDiv]

ALCL in children is usually characterized by systemic and cutaneous disease and has high response rates and good OS with doxorubicin-based combination chemotherapy.[52]

Extranodal NK-/T-cell Lymphoma

Extranodalnatural killer (NK)-/T-cell lymphoma (nasal type) is an aggressive lymphoma marked by extensive necrosis and angioinvasion, most often presenting in extranodal sites, in particular the nasal or paranasal sinus region.[53-56] Other extranodal sites include the palate, trachea, skin, and gastrointestinal tract. Hemophagocytic syndrome may occur; historically, these tumors were considered part of lethal midline granuloma.[57] In most cases, Epstein-Barr virus (EBV) genomes are detectable in the tumor cells and immunophenotyping shows CD56 positivity. Cases with blood and marrow involvement are considered NK-cell leukemia.

The increased risk of CNS involvement and of local recurrence has led to recommendations for radiation therapy locally, concurrently, or prior to the start of chemotherapy, and for intrathecal prophylaxis and/or prophylactic cranial radiation therapy.[58-63] The highly aggressive course, with poor response and short survival with standard therapies, especially for patients with advanced-stage disease or extranasal presentation, has led some investigators to recommend autologous or allogenic peripheral SCT consolidation.[54-56,64,65] L-asparaginase-containing regimens have shown anecdotal response rates greater than 50% for relapsing, refractory, or newly diagnosed stage IV patients.[66,67] NK-/T-cell lymphoma that presents only in the skin has a more favorable prognosis, especially in patients with coexpression of CD30 with CD56.[68] A benign NK-cell enteropathy (EBV negative) on endoscopic biopsy should be distinguished from NK-/T-cell lymphoma.[69]

Lymphomatoid Granulomatosis

Lymphomatoid granulomatosis is an EBV-positive large B-cell lymphoma with a predominant T-cell background.[70,71] The histology shows association with angioinvasion and vasculitis, usually manifesting as pulmonary lesions or paranasal sinus involvement.

Patients are managed like others with diffuse large cell lymphoma and require doxorubicin-based combination chemotherapy.

Angioimmunoblastic T-cell Lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) or (ATCL) was formerly called angioimmunoblastic lymphadenopathy with dysproteinemia. Characterized by clonal T-cell receptor gene rearrangement, this entity is managed like diffuse large cell lymphoma.[72-74] Patients present with profound lymphadenopathy, fever, night sweats, weight loss, skin rash, a positive Coombs test, and polyclonal hypergammaglobulinemia.[57] (Refer to the information on night sweats in the PDQ summary on Hot Flashes and Night Sweats, information on weight loss in the in the PDQ summary on Nutrition in Cancer Care, and information on skin rash in the PDQ summary on Pruritus.) Opportunistic infections are frequent because of an underlying immune deficiency. B-cell EBV genomes are detected in most affected patients.[75]

Doxorubicin-based combination chemotherapy, such as the CHOP regimen, is recommended as it is for other aggressive lymphomas.[72] The International Peripheral T-Cell Lymphoma Project involving 22 international centers identified 243 patients with AITL or ATCL; the 5-year OS and failure-free survival rates were 33% and 18%.[76] Myeloablative chemotherapy and radiation therapy with autologous or allogeneic peripheral stem cell support has been described in anecdotal reports.[64,77,78] Anecdotal responses have been reported for cyclosporine,[79] pralatrexate,[51] bendamustine,[80] and the histone deacetylase inhibitor romidepsin.[50][Level of evidence: 3iiiDiv] Occasional spontaneous remissions and protracted responses to steroids only have been reported.

Peripheral T-cell Lymphoma

Patients with peripheral T-cell lymphoma have diffuse large cell or diffuse mixed lymphoma that expresses a cell surface phenotype of a postthymic (or peripheral) T-cell expressing CD4 or CD8 but not both together.[81] Peripheral T-cell lymphoma encompasses a group of heterogeneous nodal T-cell lymphomas that will require future delineation.[57] This includes the so-called Lennert lymphoma, a T-cell lymphoma admixed with a preponderance of lymphoepithelioid cells.

Prognosis

Most investigators report worse response and survival rates for patients with peripheral T-cell lymphomas than for patients with comparably staged B-cell aggressive lymphomas.[82,83] Most patients present with multiple adverse prognostic factors (i.e., older age, stage IV, multiple extranodal sites, and elevated LDH), and these patients have a low (<20%) failure-free survival and OS at 5 years.[82,83]

Therapeutic approaches

Therapy involves doxorubicin-based combination chemotherapy (such as CHOP), which is also used for DLBCL. Consolidation using high-dose chemotherapy with autologous or allogeneic hematopoietic stem cell support has been applied to patients with advanced-stage peripheral T-cell lymphoma after induction therapy with CHOP-based regimens. Evidence for this approach is anecdotal.[64,77,84,85] For relapsing patients, pralatrexate has shown a 30% response rate and a median 10-month duration of response for 109 evaluable patients in a prospective trial.[50,86][Level of evidence: 3iiiDiv] Also for relapsing patients, similar response rates were seen for romidepsin for 130 evaluable patients in a prospective trial.[50][Level of evidence: 3iiiDiv] Anecdotal responses have been seen with pralatrexate [51] and with bendamustine.[80][Level of evidence: 3iiiDiv] Anecdotal responses have also been seen with alemtuzumab, an anti-CD52 monoclonal antibody, or denileukin diftitox, a toxin-antibody ligand, after relapse from previous chemotherapy.[87,88]

An unusual type of peripheral T-cell lymphoma occurring mostly in young men, hepatosplenic T-cell lymphoma, appears to be localized to the hepatic and splenic sinusoids, with cell surface expression of the T-cell receptor gamma/delta.[89-93] Another variant, subcutaneous panniculitis-like T-cell lymphoma, is localized to subcutaneous tissue associated with hemophagocytic syndrome.[94-97] These patients have cells that express alpha-beta phenotype. Those with gamma-delta phenotype have a more aggressive clinical course and are classified as cutaneous gamma-delta T-cell lymphoma.[98-100] These patients may manifest involvement of the epidermis, dermis, subcutaneous region, or mucosa. These entities have extremely poor prognoses with an extremely aggressive clinical course and are treated within the same paradigm as the highest-risk groups with DLBCL.[64]

Enteropathy-type Intestinal T-cell Lymphoma

Enteropathy-type intestinal T-cell lymphoma involves the small bowel of patients with gluten-sensitive enteropathy (celiac sprue).[57,101-103] Since a gluten-free diet prevents the development of lymphoma, patients diagnosed with celiac sprue in childhood rarely develop lymphoma. The diagnosis of celiac disease is usually made by finding villous atrophy in the resected intestine. Surgery is often required for diagnosis and to avoid perforation during therapy.

Therapy is with doxorubicin-based combination chemotherapy, but relapse rates appear higher than for comparably staged diffuse large cell lymphoma.[102-104] Complications of treatment include gastrointestinal bleeding, small bowel perforation, and enterocolic fistulae; patients often require parenteral nutrition. (Refer to the PDQ summaries on Gastrointestinal Complications and Nutrition in Cancer Care for more information on parenteral nutrition.) Multifocal intestinal perforations and visceral abdominal involvement are seen at the time of relapse. High-dose therapy with hematopoietic stem cell rescue has been applied in first remission or at relapse.[64,102,105][Level of evidence: 3iiiDiii] Evidence for this approach is anecdotal.

Intravascular Large B-cell Lymphoma (Intravascular Lymphomatosis)

Intravascular lymphomatosis is characterized by large cell lymphoma confined to the intravascular lumen. The brain, kidneys, lungs, and skin are the organs most likely affected by intravascular lymphomatosis.

With the use of aggressive combination chemotherapy, the prognosis is similar to more conventional presentations.[106,107]

Burkitt Lymphoma/Diffuse Small Noncleaved-cell Lymphoma

Burkitt lymphoma/diffuse small noncleaved-cell lymphoma typically involves younger patients and represents the most common type of pediatric NHL.[108] These types of aggressive extranodal B-cell lymphomas are characterized by translocation and deregulation of the C-myc gene on chromosome 8.[109] A subgroup of patients with dual translocation of C-myc and bcl-2 appear to have an extremely poor outcome despite aggressive therapy (5-month OS).[110][Level of evidence: 3iiiA]

In some patients with larger B cells, there is morphologic overlap with DLBCL. These Burkitt-like large cell lymphomas show C-myc deregulation, extremely high proliferation rates, and a gene-expression profile as expected for classic Burkitt lymphoma.[111-113] Endemic cases, usually from Africa, involve the facial bones or jaws of children, mostly containing EBV genomes. Sporadic cases usually involve the gastrointestinal system, ovaries, or kidneys. Patients present with rapidly growing masses and a very high LDH but are potentially curable with intensive doxorubicin-based combination chemotherapy.

Therapeutic approaches

Treatment of Burkitt lymphoma/diffuse small noncleaved-cell lymphoma involves aggressive multidrug regimens similar to those used for the advanced-stage aggressive lymphomas (diffuse large cell).[114-116] Aggressive combination chemotherapy, which is patterned after that used in childhood Burkitt lymphoma, has been described in CLB-9251 (NCT00002494), for example, and has been very successful for adult patients with more than 60% of advanced-stage patients free of disease at 5 years.[117-122] Adverse prognostic factors include bulky abdominal disease and high serum LDH. In some institutions, treatment includes the use of consolidative bone marrow transplantation (BMT).[123,124] Patients with Burkitt lymphoma have a 20% to 30% lifetime risk of CNS involvement. Prophylaxis with intrathecal chemotherapy is required as part of induction therapy.[125] (Refer to the PDQ summaries on Primary CNS Lymphoma Treatment and AIDS-Related Lymphoma Treatment for more information.)

Lymphoblastic Lymphoma

Lymphoblastic lymphoma (precursor T-cell) is a very aggressive form of NHL. It often occurs in young patients but not exclusively.[126] It is commonly associated with large mediastinal masses and has a high predilection for disseminating to bone marrow and to the CNS.

Treatment is usually patterned after that for acute lymphoblastic leukemia. Intensive combination chemotherapy with or without BMT is the standard treatment of this aggressive histologic type of NHL.[127-129] Radiation therapy is sometimes given to areas of bulky tumor masses. Because these forms of NHL tend to progress quickly, combination chemotherapy is instituted rapidly once the diagnosis has been confirmed. Careful review of the pathologic specimens, bone marrow aspirate, biopsy specimen, cerebrospinal fluid cytology, and lymphocyte marker constitute the most important aspects of the pretreatment staging workup. (Refer to the PDQ summary on Adult Acute Lymphoblastic Leukemia Treatment for more information.)

Adult T-cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma (ATL) is caused by infection with the retrovirus human T-lymphotrophic virus 1 and is frequently associated with lymphadenopathy, hypercalcemia, circulating leukemic cells, bone and skin involvement, hepatosplenomegaly, a rapidly progressive course, and poor response to combination chemotherapy.[130,131] ATL has been divided into four clinical subtypes: acute, lymphoma, chronic, and smoldering.[132,133]

The acute and lymphoma types of ATL have done poorly with strategies of combination chemotherapy and allogeneic stem cell transplantation (ASCT) with a median OS under 1 year.[134-136] Using combination chemotherapy, less than 10%of 807 patients were alive after 4 years.[136] Anecdotal durable remissions have been reported after ASCT and even after subsequent donor lymphocyte infusion for relapses after transplant.[137][Level of evidence: 3iiiDiv] Among 585 patients who underwent ASCT, the 3-year OS was 36%.[138][Level of evidence: 3iiiA]

The combination of zidovudine and interferon-alpha has activity against ATL, even for patients who failed previous cytotoxic therapy. Durable remissions are seen in the majority of presenting patients with this combination but are not seen in patients with the lymphoma subtype of ATL.[139-143] Symptomatic local progression for all subtypes responds well to palliative radiation therapy.[144]

Mantle Cell Lymphoma

Mantle cell lymphoma is found in lymph nodes, the spleen, bone marrow, blood, and sometimes the gastrointestinal system (lymphomatous polyposis).[145-147] Mantle cell lymphoma is characterized by CD5-positive follicular mantle B cells, a translocation of chromosomes 11 and 14, and an overexpression of the cyclin D1 protein.[148,149]

Like the low-grade lymphomas, mantle cell lymphoma appears incurable with anthracycline-based chemotherapy and occurs in older patients with generally asymptomatic advanced-stage disease.[150] The median survival, however, is significantly shorter (3–5 years) than that of other lymphomas, and this histology is now considered to be an aggressive lymphoma.[151] A diffuse pattern and the blastoid variant have an aggressive course with shorter survival, while the mantle zone type may have a more indolent course.[152,153] A high cell-proliferation rate (increased Ki-67, mitotic index, beta-2-microglobulin) may be associated with a poorer prognosis.[148,154]

Therapeutic approaches

It is unclear which chemotherapeutic approach offers the best long-term survival in this clinicopathologic entity; early refractoriness to chemotherapy is a usual feature.[155-159] In a prospective randomized trial, 532 patients older than 60 years and not eligible for SCT were given either R-CHOP or R-FC (rituximab, fludarabine, cyclophosphamide) for 6 to 8 cycles, followed by maintenance therapy in responders randomly assigned to rituximab or interferon alfa maintenance therapy.[160] With a median follow-up of 37 months, the OS was significantly shorter after R-FC than after R-CHOP (47% vs. 62%, P = .005; hazard ratio [HR]death, 1.50; 95% CI, 1.13–1.99).[160][Level of evidence: 1iiA] Event-free survival favored rituximab over interferon alfa (57% progression-free survival at 4 years vs. 34%, P = .01; HR, 0.55; 95% CI, 0.36–0.87), but OS did not differ significantly (79% vs. 67% at 4 years, P = .13).[160][Level of evidence: 1iiDi] However, patients who received R-CHOP induction showed an OS benefit for rituximab over interferon alfa (87% vs. 63% at 4 years, P = .005).[160][Level of evidence: 3iiiA] Patients with low risk on the IPI may do well when initial therapy is deferred.[161][Level of evidence: 3iiiDiv] Many investigators are exploring high-dose chemoradioimmunotherapy with stem cell/marrow support or nonmyeloablative ASCT.[159,155,156,162-171] Thus far, randomized trials have not shown OS benefits from these newer approaches.[168] Bortezomib shows response rates close to 50% in relapsed patients, prompting clinical trials combining this proteasome inhibitor with rituximab and cytotoxic agents in first-line therapy.[172,173][Level of evidence: 3iiiDiv]

Polymorphic Posttransplantation Lymphoproliferative Disorder (PTLD)

Patients who undergo transplantation of the heart, lung, liver, kidney, or pancreas usually require lifelong immunosuppression. This may result in PTLD in 1% to 3% of recipients, which appears as an aggressive lymphoma.[174] Pathologists can distinguish a polyclonal B-cell hyperplasia from a monoclonal B-cell lymphoma; both are almost always associated with EBV.[175]

Prognosis

Poor performance status, grafted organ involvement, high IPI, elevated LDH, and multiple sites of disease are poor prognostic factors for PTLD.[176,177]

Therapeutic options

In some cases, withdrawal of immunosuppression results in eradication of the lymphoma.[178,179] When this is unsuccessful or not feasible, a trial of rituximab may be considered, because it has shown durable remissions in approximately 60% of patients and a favorable toxicity profile.[179,180] Sometimes, a combination of acyclovir and interferon-alpha has been used.[174,181] If these measures fail, doxorubicin-based combination chemotherapy is recommended, although most patients can avoid cytotoxic therapy.[182] Localized presentations can be controlled with surgery or radiation therapy alone. These localized mass lesions, which may grow over a period of months, are often phenotypically polyclonal and tend to occur within weeks or a few months after transplantation.[175] Multifocal, rapidly progressive disease occurs late after transplantation (>1 year) and is usually phenotypically monoclonal and associated with EBV.[183] These patients may have durable remissions using standard chemotherapy regimens for aggressive lymphoma.[183-185] Instances of EBV-negative PTLD occur even later (median, 5 years posttransplant) and have particularly poor prognoses.[186] A sustained clinical response after failure from chemotherapy was attained using an immunotoxin (anti-CD22 B-cell surface antigen antibody linked with ricin, a plant toxin).[187] An anti-interleukin-6 monoclonal antibody is also under clinical evaluation.[188]

True Histiocytic Lymphoma

True histiocytic lymphomas are very rare tumors that show histiocytic differentiation and express histiocytic markers in the absence of B-cell or T-cell lineage-specific immunologic markers.[189,190] Care must be taken with immunophenotypic tests to exclude ALCL or hemophagocytic syndromes caused by viral infections, especially EBV.

Therapeutic options

Therapy is modeled after the treatment of comparably staged diffuse large cell lymphomas, but the optimal approach remains to be defined.

Primary Effusion Lymphoma

Primary effusion lymphoma presents exclusively or mainly in the pleural, pericardial, or abdominal cavities in the absence of an identifiable tumor mass.[191] Patients are usually human immunodeficiency virus seropositive, and the tumor usually contains Kaposi sarcoma–associated herpes virus/human herpes virus 8.

Prognosis

The prognosis of primary effusion lymphoma is extremely poor.

Therapeutic approaches

Therapy is usually modeled after the treatment of comparably staged diffuse large cell lymphomas.

References
  1. Armitage JO, Weisenburger DD: New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project. J Clin Oncol 16 (8): 2780-95, 1998.  [PUBMED Abstract]

  2. Delabie J, Vandenberghe E, Kennes C, et al.: Histiocyte-rich B-cell lymphoma. A distinct clinicopathologic entity possibly related to lymphocyte predominant Hodgkin's disease, paragranuloma subtype. Am J Surg Pathol 16 (1): 37-48, 1992.  [PUBMED Abstract]

  3. Achten R, Verhoef G, Vanuytsel L, et al.: T-cell/histiocyte-rich large B-cell lymphoma: a distinct clinicopathologic entity. J Clin Oncol 20 (5): 1269-77, 2002.  [PUBMED Abstract]

  4. Bouabdallah R, Mounier N, Guettier C, et al.: T-cell/histiocyte-rich large B-cell lymphomas and classical diffuse large B-cell lymphomas have similar outcome after chemotherapy: a matched-control analysis. J Clin Oncol 21 (7): 1271-7, 2003.  [PUBMED Abstract]

  5. Ghesquières H, Berger F, Felman P, et al.: Clinicopathologic characteristics and outcome of diffuse large B-cell lymphomas presenting with an associated low-grade component at diagnosis. J Clin Oncol 24 (33): 5234-41, 2006.  [PUBMED Abstract]

  6. Miller TP, Dahlberg S, Cassady JR, et al.: Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin's lymphoma. N Engl J Med 339 (1): 21-6, 1998.  [PUBMED Abstract]

  7. Coiffier B, Lepage E, Briere J, et al.: CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med 346 (4): 235-42, 2002.  [PUBMED Abstract]

  8. Coiffier B: State-of-the-art therapeutics: diffuse large B-cell lymphoma. J Clin Oncol 23 (26): 6387-93, 2005.  [PUBMED Abstract]

  9. Habermann TM, Weller EA, Morrison VA, et al.: Rituximab-CHOP versus CHOP alone or with maintenance rituximab in older patients with diffuse large B-cell lymphoma. J Clin Oncol 24 (19): 3121-7, 2006.  [PUBMED Abstract]

  10. A predictive model for aggressive non-Hodgkin's lymphoma. The International Non-Hodgkin's Lymphoma Prognostic Factors Project. N Engl J Med 329 (14): 987-94, 1993.  [PUBMED Abstract]

  11. Møller MB, Christensen BE, Pedersen NT: Prognosis of localized diffuse large B-cell lymphoma in younger patients. Cancer 98 (3): 516-21, 2003.  [PUBMED Abstract]

  12. Cuccuini W, Briere J, Mounier N, et al.: MYC+ diffuse large B-cell lymphoma is not salvaged by classical R-ICE or R-DHAP followed by BEAM plus autologous stem cell transplantation. Blood 119 (20): 4619-24, 2012.  [PUBMED Abstract]

  13. Johnson NA, Slack GW, Savage KJ, et al.: Concurrent expression of MYC and BCL2 in diffuse large B-cell lymphoma treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. J Clin Oncol 30 (28): 3452-9, 2012.  [PUBMED Abstract]

  14. Green TM, Young KH, Visco C, et al.: Immunohistochemical double-hit score is a strong predictor of outcome in patients with diffuse large B-cell lymphoma treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. J Clin Oncol 30 (28): 3460-7, 2012.  [PUBMED Abstract]

  15. Horn H, Ziepert M, Becher C, et al.: MYC status in concert with BCL2 and BCL6 expression predicts outcome in diffuse large B-cell lymphoma. Blood 121 (12): 2253-63, 2013.  [PUBMED Abstract]

  16. Canellos GP: CHOP may have been part of the beginning but certainly not the end: issues in risk-related therapy of large-cell lymphoma. J Clin Oncol 15 (5): 1713-6, 1997.  [PUBMED Abstract]

  17. Lossos IS, Czerwinski DK, Alizadeh AA, et al.: Prediction of survival in diffuse large-B-cell lymphoma based on the expression of six genes. N Engl J Med 350 (18): 1828-37, 2004.  [PUBMED Abstract]

  18. Abramson JS, Shipp MA: Advances in the biology and therapy of diffuse large B-cell lymphoma: moving toward a molecularly targeted approach. Blood 106 (4): 1164-74, 2005.  [PUBMED Abstract]

  19. de Jong D, Rosenwald A, Chhanabhai M, et al.: Immunohistochemical prognostic markers in diffuse large B-cell lymphoma: validation of tissue microarray as a prerequisite for broad clinical applications--a study from the Lunenburg Lymphoma Biomarker Consortium. J Clin Oncol 25 (7): 805-12, 2007.  [PUBMED Abstract]

  20. Fu K, Weisenburger DD, Choi WW, et al.: Addition of rituximab to standard chemotherapy improves the survival of both the germinal center B-cell-like and non-germinal center B-cell-like subtypes of diffuse large B-cell lymphoma. J Clin Oncol 26 (28): 4587-94, 2008.  [PUBMED Abstract]

  21. Lenz G, Staudt LM: Aggressive lymphomas. N Engl J Med 362 (15): 1417-29, 2010.  [PUBMED Abstract]

  22. Glantz MJ, Cole BF, Recht L, et al.: High-dose intravenous methotrexate for patients with nonleukemic leptomeningeal cancer: is intrathecal chemotherapy necessary? J Clin Oncol 16 (4): 1561-7, 1998.  [PUBMED Abstract]

  23. Fisher RI, Gaynor ER, Dahlberg S, et al.: Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin's lymphoma. N Engl J Med 328 (14): 1002-6, 1993.  [PUBMED Abstract]

  24. Bernstein SH, Unger JM, Leblanc M, et al.: Natural history of CNS relapse in patients with aggressive non-Hodgkin's lymphoma: a 20-year follow-up analysis of SWOG 8516 -- the Southwest Oncology Group. J Clin Oncol 27 (1): 114-9, 2009.  [PUBMED Abstract]

  25. van Besien K, Ha CS, Murphy S, et al.: Risk factors, treatment, and outcome of central nervous system recurrence in adults with intermediate-grade and immunoblastic lymphoma. Blood 91 (4): 1178-84, 1998.  [PUBMED Abstract]

  26. Villa D, Connors JM, Shenkier TN, et al.: Incidence and risk factors for central nervous system relapse in patients with diffuse large B-cell lymphoma: the impact of the addition of rituximab to CHOP chemotherapy. Ann Oncol 21 (5): 1046-52, 2010.  [PUBMED Abstract]

  27. Boehme V, Schmitz N, Zeynalova S, et al.: CNS events in elderly patients with aggressive lymphoma treated with modern chemotherapy (CHOP-14) with or without rituximab: an analysis of patients treated in the RICOVER-60 trial of the German High-Grade Non-Hodgkin Lymphoma Study Group (DSHNHL). Blood 113 (17): 3896-902, 2009.  [PUBMED Abstract]

  28. Lazzarino M, Orlandi E, Paulli M, et al.: Primary mediastinal B-cell lymphoma with sclerosis: an aggressive tumor with distinctive clinical and pathologic features. J Clin Oncol 11 (12): 2306-13, 1993.  [PUBMED Abstract]

  29. Kirn D, Mauch P, Shaffer K, et al.: Large-cell and immunoblastic lymphoma of the mediastinum: prognostic features and treatment outcome in 57 patients. J Clin Oncol 11 (7): 1336-43, 1993.  [PUBMED Abstract]

  30. Aisenberg AC: Primary large-cell lymphoma of the mediastinum. J Clin Oncol 11 (12): 2291-4, 1993.  [PUBMED Abstract]

  31. Abou-Elella AA, Weisenburger DD, Vose JM, et al.: Primary mediastinal large B-cell lymphoma: a clinicopathologic study of 43 patients from the Nebraska Lymphoma Study Group. J Clin Oncol 17 (3): 784-90, 1999.  [PUBMED Abstract]

  32. Cazals-Hatem D, Lepage E, Brice P, et al.: Primary mediastinal large B-cell lymphoma. A clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA ("Groupe d'Etude des Lymphomes de l'Adulte") study. Am J Surg Pathol 20 (7): 877-88, 1996.  [PUBMED Abstract]

  33. Popat U, Przepiork D, Champlin R, et al.: High-dose chemotherapy for relapsed and refractory diffuse large B-cell lymphoma: mediastinal localization predicts for a favorable outcome. J Clin Oncol 16 (1): 63-9, 1998.  [PUBMED Abstract]

  34. van Besien K, Kelta M, Bahaguna P: Primary mediastinal B-cell lymphoma: a review of pathology and management. J Clin Oncol 19 (6): 1855-64, 2001.  [PUBMED Abstract]

  35. Dunleavy K, Pittaluga S, Maeda LS, et al.: Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. N Engl J Med 368 (15): 1408-16, 2013.  [PUBMED Abstract]

  36. Savage KJ, Al-Rajhi N, Voss N, et al.: Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann Oncol 17 (1): 123-30, 2006.  [PUBMED Abstract]

  37. Vassilakopoulos TP, Pangalis GA, Katsigiannis A, et al.: Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone with or without radiotherapy in primary mediastinal large B-cell lymphoma: the emerging standard of care. Oncologist 17 (2): 239-49, 2012.  [PUBMED Abstract]

  38. Rieger M, Osterborg A, Pettengell R, et al.: Primary mediastinal B-cell lymphoma treated with CHOP-like chemotherapy with or without rituximab: results of the Mabthera International Trial Group study. Ann Oncol 22 (3): 664-70, 2011.  [PUBMED Abstract]

  39. Longo DL: What's the deal with follicular lymphomas? J Clin Oncol 11 (2): 202-8, 1993.  [PUBMED Abstract]

  40. Anderson JR, Vose JM, Bierman PJ, et al.: Clinical features and prognosis of follicular large-cell lymphoma: a report from the Nebraska Lymphoma Study Group. J Clin Oncol 11 (2): 218-24, 1993.  [PUBMED Abstract]

  41. Bartlett NL, Rizeq M, Dorfman RF, et al.: Follicular large-cell lymphoma: intermediate or low grade? J Clin Oncol 12 (7): 1349-57, 1994.  [PUBMED Abstract]

  42. Wendum D, Sebban C, Gaulard P, et al.: Follicular large-cell lymphoma treated with intensive chemotherapy: an analysis of 89 cases included in the LNH87 trial and comparison with the outcome of diffuse large B-cell lymphoma. Groupe d'Etude des Lymphomes de l'Adulte. J Clin Oncol 15 (4): 1654-63, 1997.  [PUBMED Abstract]

  43. Hans CP, Weisenburger DD, Vose JM, et al.: A significant diffuse component predicts for inferior survival in grade 3 follicular lymphoma, but cytologic subtypes do not predict survival. Blood 101 (6): 2363-7, 2003.  [PUBMED Abstract]

  44. Vose JM, Bierman PJ, Lynch JC, et al.: Effect of follicularity on autologous transplantation for large-cell non-Hodgkin's lymphoma. J Clin Oncol 16 (3): 844-9, 1998.  [PUBMED Abstract]

  45. Bai RY, Ouyang T, Miething C, et al.: Nucleophosmin-anaplastic lymphoma kinase associated with anaplastic large-cell lymphoma activates the phosphatidylinositol 3-kinase/Akt antiapoptotic signaling pathway. Blood 96 (13): 4319-27, 2000.  [PUBMED Abstract]

  46. Gascoyne RD, Aoun P, Wu D, et al.: Prognostic significance of anaplastic lymphoma kinase (ALK) protein expression in adults with anaplastic large cell lymphoma. Blood 93 (11): 3913-21, 1999.  [PUBMED Abstract]

  47. Sibon D, Fournier M, Brière J, et al.: Long-term outcome of adults with systemic anaplastic large-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte trials. J Clin Oncol 30 (32): 3939-46, 2012.  [PUBMED Abstract]

  48. Younes A, Bartlett NL, Leonard JP, et al.: Brentuximab vedotin (SGN-35) for relapsed CD30-positive lymphomas. N Engl J Med 363 (19): 1812-21, 2010.  [PUBMED Abstract]

  49. Pro B, Advani R, Brice P, et al.: Brentuximab vedotin (SGN-35) in patients with relapsed or refractory systemic anaplastic large-cell lymphoma: results of a phase II study. J Clin Oncol 30 (18): 2190-6, 2012.  [PUBMED Abstract]

  50. Coiffier B, Pro B, Prince HM, et al.: Results from a pivotal, open-label, phase II study of romidepsin in relapsed or refractory peripheral T-cell lymphoma after prior systemic therapy. J Clin Oncol 30 (6): 631-6, 2012.  [PUBMED Abstract]

  51. O'Connor OA, Horwitz S, Hamlin P, et al.: Phase II-I-II study of two different doses and schedules of pralatrexate, a high-affinity substrate for the reduced folate carrier, in patients with relapsed or refractory lymphoma reveals marked activity in T-cell malignancies. J Clin Oncol 27 (26): 4357-64, 2009.  [PUBMED Abstract]

  52. Seidemann K, Tiemann M, Schrappe M, et al.: Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 97 (12): 3699-706, 2001.  [PUBMED Abstract]

  53. Lipford EH Jr, Margolick JB, Longo DL, et al.: Angiocentric immunoproliferative lesions: a clinicopathologic spectrum of post-thymic T-cell proliferations. Blood 72 (5): 1674-81, 1988.  [PUBMED Abstract]

  54. Liang R, Todd D, Chan TK, et al.: Treatment outcome and prognostic factors for primary nasal lymphoma. J Clin Oncol 13 (3): 666-70, 1995.  [PUBMED Abstract]

  55. Cheung MM, Chan JK, Lau WH, et al.: Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients. J Clin Oncol 16 (1): 70-7, 1998.  [PUBMED Abstract]

  56. Hausdorff J, Davis E, Long G, et al.: Non-Hodgkin's lymphoma of the paranasal sinuses: clinical and pathological features, and response to combined-modality therapy. Cancer J Sci Am 3 (5): 303-11, 1997 Sep-Oct.  [PUBMED Abstract]

  57. Rizvi MA, Evens AM, Tallman MS, et al.: T-cell non-Hodgkin lymphoma. Blood 107 (4): 1255-64, 2006.  [PUBMED Abstract]

  58. Li YX, Yao B, Jin J, et al.: Radiotherapy as primary treatment for stage IE and IIE nasal natural killer/T-cell lymphoma. J Clin Oncol 24 (1): 181-9, 2006.  [PUBMED Abstract]

  59. Lee J, Suh C, Park YH, et al.: Extranodal natural killer T-cell lymphoma, nasal-type: a prognostic model from a retrospective multicenter study. J Clin Oncol 24 (4): 612-8, 2006.  [PUBMED Abstract]

  60. Li CC, Tien HF, Tang JL, et al.: Treatment outcome and pattern of failure in 77 patients with sinonasal natural killer/T-cell or T-cell lymphoma. Cancer 100 (2): 366-75, 2004.  [PUBMED Abstract]

  61. Yamaguchi M, Tobinai K, Oguchi M, et al.: Phase I/II study of concurrent chemoradiotherapy for localized nasal natural killer/T-cell lymphoma: Japan Clinical Oncology Group Study JCOG0211. J Clin Oncol 27 (33): 5594-600, 2009.  [PUBMED Abstract]

  62. Kim SJ, Kim K, Kim BS, et al.: Phase II trial of concurrent radiation and weekly cisplatin followed by VIPD chemotherapy in newly diagnosed, stage IE to IIE, nasal, extranodal NK/T-Cell Lymphoma: Consortium for Improving Survival of Lymphoma study. J Clin Oncol 27 (35): 6027-32, 2009.  [PUBMED Abstract]

  63. Li YX, Fang H, Liu QF, et al.: Clinical features and treatment outcome of nasal-type NK/T-cell lymphoma of Waldeyer ring. Blood 112 (8): 3057-64, 2008.  [PUBMED Abstract]

  64. Le Gouill S, Milpied N, Buzyn A, et al.: Graft-versus-lymphoma effect for aggressive T-cell lymphomas in adults: a study by the Société Francaise de Greffe de Moëlle et de Thérapie Cellulaire. J Clin Oncol 26 (14): 2264-71, 2008.  [PUBMED Abstract]

  65. Au WY, Weisenburger DD, Intragumtornchai T, et al.: Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood 113 (17): 3931-7, 2009.  [PUBMED Abstract]

  66. Jaccard A, Gachard N, Marin B, et al.: Efficacy of L-asparaginase with methotrexate and dexamethasone (AspaMetDex regimen) in patients with refractory or relapsing extranodal NK/T-cell lymphoma, a phase 2 study. Blood 117 (6): 1834-9, 2011.  [PUBMED Abstract]

  67. Yamaguchi M, Kwong YL, Kim WS, et al.: Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol 29 (33): 4410-6, 2011.  [PUBMED Abstract]

  68. Mraz-Gernhard S, Natkunam Y, Hoppe RT, et al.: Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course. J Clin Oncol 19 (8): 2179-88, 2001.  [PUBMED Abstract]

  69. Mansoor A, Pittaluga S, Beck PL, et al.: NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series. Blood 117 (5): 1447-52, 2011.  [PUBMED Abstract]

  70. Guinee D Jr, Jaffe E, Kingma D, et al.: Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis. Am J Surg Pathol 18 (8): 753-64, 1994.  [PUBMED Abstract]

  71. Myers JL, Kurtin PJ, Katzenstein AL, et al.: Lymphomatoid granulomatosis. Evidence of immunophenotypic diversity and relationship to Epstein-Barr virus infection. Am J Surg Pathol 19 (11): 1300-12, 1995.  [PUBMED Abstract]

  72. Siegert W, Agthe A, Griesser H, et al.: Treatment of angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma using prednisone with or without the COPBLAM/IMVP-16 regimen. A multicenter study. Kiel Lymphoma Study Group. Ann Intern Med 117 (5): 364-70, 1992.  [PUBMED Abstract]

  73. Jaffe ES: Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains. Ann Oncol 6 (7): 631-2, 1995.  [PUBMED Abstract]

  74. Siegert W, Nerl C, Agthe A, et al.: Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group. Ann Oncol 6 (7): 659-64, 1995.  [PUBMED Abstract]

  75. Bräuninger A, Spieker T, Willenbrock K, et al.: Survival and clonal expansion of mutating "forbidden" (immunoglobulin receptor-deficient) epstein-barr virus-infected b cells in angioimmunoblastic t cell lymphoma. J Exp Med 194 (7): 927-40, 2001.  [PUBMED Abstract]

  76. Federico M, Rudiger T, Bellei M, et al.: Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project. J Clin Oncol 31 (2): 240-6, 2013.  [PUBMED Abstract]

  77. Reimer P, Rüdiger T, Geissinger E, et al.: Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multicenter study. J Clin Oncol 27 (1): 106-13, 2009.  [PUBMED Abstract]

  78. Kyriakou C, Canals C, Finke J, et al.: Allogeneic stem cell transplantation is able to induce long-term remissions in angioimmunoblastic T-cell lymphoma: a retrospective study from the lymphoma working party of the European group for blood and marrow transplantation. J Clin Oncol 27 (24): 3951-8, 2009.  [PUBMED Abstract]

  79. Advani R, Horwitz S, Zelenetz A, et al.: Angioimmunoblastic T cell lymphoma: treatment experience with cyclosporine. Leuk Lymphoma 48 (3): 521-5, 2007.  [PUBMED Abstract]

  80. Damaj G, Gressin R, Bouabdallah K, et al.: Results from a prospective, open-label, phase II trial of bendamustine in refractory or relapsed T-cell lymphomas: the BENTLY trial. J Clin Oncol 31 (1): 104-10, 2013.  [PUBMED Abstract]

  81. Rüdiger T, Weisenburger DD, Anderson JR, et al.: Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin's Lymphoma Classification Project. Ann Oncol 13 (1): 140-9, 2002.  [PUBMED Abstract]

  82. Weisenburger DD, Savage KJ, Harris NL, et al.: Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood 117 (12): 3402-8, 2011.  [PUBMED Abstract]

  83. Sonnen R, Schmidt WP, Müller-Hermelink HK, et al.: The International Prognostic Index determines the outcome of patients with nodal mature T-cell lymphomas. Br J Haematol 129 (3): 366-72, 2005.  [PUBMED Abstract]

  84. Rodriguez J, Munsell M, Yazji S, et al.: Impact of high-dose chemotherapy on peripheral T-cell lymphomas. J Clin Oncol 19 (17): 3766-70, 2001.  [PUBMED Abstract]

  85. d'Amore F, Relander T, Lauritzsen GF, et al.: Up-front autologous stem-cell transplantation in peripheral T-cell lymphoma: NLG-T-01. J Clin Oncol 30 (25): 3093-9, 2012.  [PUBMED Abstract]

  86. O'Connor OA, Pro B, Pinter-Brown L, et al.: Pralatrexate in patients with relapsed or refractory peripheral T-cell lymphoma: results from the pivotal PROPEL study. J Clin Oncol 29 (9): 1182-9, 2011.  [PUBMED Abstract]

  87. Enblad G, Hagberg H, Erlanson M, et al.: A pilot study of alemtuzumab (anti-CD52 monoclonal antibody) therapy for patients with relapsed or chemotherapy-refractory peripheral T-cell lymphomas. Blood 103 (8): 2920-4, 2004.  [PUBMED Abstract]

  88. Talpur R, Apisarnthanarax N, Ward S, et al.: Treatment of refractory peripheral T-cell lymphoma with denileukin diftitox (ONTAK). Leuk Lymphoma 43 (1): 121-6, 2002.  [PUBMED Abstract]

  89. Farcet JP, Gaulard P, Marolleau JP, et al.: Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta. Blood 75 (11): 2213-9, 1990.  [PUBMED Abstract]

  90. Wong KF, Chan JK, Matutes E, et al.: Hepatosplenic gamma delta T-cell lymphoma. A distinctive aggressive lymphoma type. Am J Surg Pathol 19 (6): 718-26, 1995.  [PUBMED Abstract]

  91. François A, Lesesve JF, Stamatoullas A, et al.: Hepatosplenic gamma/delta T-cell lymphoma: a report of two cases in immunocompromised patients, associated with isochromosome 7q. Am J Surg Pathol 21 (7): 781-90, 1997.  [PUBMED Abstract]

  92. Belhadj K, Reyes F, Farcet JP, et al.: Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood 102 (13): 4261-9, 2003.  [PUBMED Abstract]

  93. Chanan-Khan A, Islam T, Alam A, et al.: Long-term survival with allogeneic stem cell transplant and donor lymphocyte infusion following salvage therapy with anti-CD52 monoclonal antibody (Campath) in a patient with alpha/beta hepatosplenic T-cell non-Hodgkin's lymphoma. Leuk Lymphoma 45 (8): 1673-5, 2004.  [PUBMED Abstract]

  94. Go RS, Wester SM: Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: a systematic analysis of 156 patients reported in the literature. Cancer 101 (6): 1404-13, 2004.  [PUBMED Abstract]

  95. Marzano AV, Berti E, Paulli M, et al.: Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol 136 (7): 889-96, 2000.  [PUBMED Abstract]

  96. Hoque SR, Child FJ, Whittaker SJ, et al.: Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol 148 (3): 516-25, 2003.  [PUBMED Abstract]

  97. Salhany KE, Macon WR, Choi JK, et al.: Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol 22 (7): 881-93, 1998.  [PUBMED Abstract]

  98. Massone C, Chott A, Metze D, et al.: Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients. Am J Surg Pathol 28 (6): 719-35, 2004.  [PUBMED Abstract]

  99. Arnulf B, Copie-Bergman C, Delfau-Larue MH, et al.: Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood 91 (5): 1723-31, 1998.  [PUBMED Abstract]

  100. Toro JR, Liewehr DJ, Pabby N, et al.: Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood 101 (9): 3407-12, 2003.  [PUBMED Abstract]

  101. Egan LJ, Walsh SV, Stevens FM, et al.: Celiac-associated lymphoma. A single institution experience of 30 cases in the combination chemotherapy era. J Clin Gastroenterol 21 (2): 123-9, 1995.  [PUBMED Abstract]

  102. Gale J, Simmonds PD, Mead GM, et al.: Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol 18 (4): 795-803, 2000.  [PUBMED Abstract]

  103. Di Sabatino A, Biagi F, Gobbi PG, et al.: How I treat enteropathy-associated T-cell lymphoma. Blood 119 (11): 2458-68, 2012.  [PUBMED Abstract]

  104. Daum S, Ullrich R, Heise W, et al.: Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma. J Clin Oncol 21 (14): 2740-6, 2003.  [PUBMED Abstract]

  105. Sieniawski M, Angamuthu N, Boyd K, et al.: Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood 115 (18): 3664-70, 2010.  [PUBMED Abstract]

  106. Shimada K, Matsue K, Yamamoto K, et al.: Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 26 (19): 3189-95, 2008.  [PUBMED Abstract]

  107. Ponzoni M, Ferreri AJ, Campo E, et al.: Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol 25 (21): 3168-73, 2007.  [PUBMED Abstract]

  108. Blum KA, Lozanski G, Byrd JC: Adult Burkitt leukemia and lymphoma. Blood 104 (10): 3009-20, 2004.  [PUBMED Abstract]

  109. Onciu M, Schlette E, Zhou Y, et al.: Secondary chromosomal abnormalities predict outcome in pediatric and adult high-stage Burkitt lymphoma. Cancer 107 (5): 1084-92, 2006.  [PUBMED Abstract]

  110. Macpherson N, Lesack D, Klasa R, et al.: Small noncleaved, non-Burkitt's (Burkit-Like) lymphoma: cytogenetics predict outcome and reflect clinical presentation. J Clin Oncol 17 (5): 1558-67, 1999.  [PUBMED Abstract]

  111. Dave SS, Fu K, Wright GW, et al.: Molecular diagnosis of Burkitt's lymphoma. N Engl J Med 354 (23): 2431-42, 2006.  [PUBMED Abstract]

  112. Hummel M, Bentink S, Berger H, et al.: A biologic definition of Burkitt's lymphoma from transcriptional and genomic profiling. N Engl J Med 354 (23): 2419-30, 2006.  [PUBMED Abstract]

  113. Salaverria I, Siebert R: The gray zone between Burkitt's lymphoma and diffuse large B-cell lymphoma from a genetics perspective. J Clin Oncol 29 (14): 1835-43, 2011.  [PUBMED Abstract]

  114. Longo DL, Duffey PL, Jaffe ES, et al.: Diffuse small noncleaved-cell, non-Burkitt's lymphoma in adults: a high-grade lymphoma responsive to ProMACE-based combination chemotherapy. J Clin Oncol 12 (10): 2153-9, 1994.  [PUBMED Abstract]

  115. McMaster ML, Greer JP, Greco FA, et al.: Effective treatment of small-noncleaved-cell lymphoma with high-intensity, brief-duration chemotherapy. J Clin Oncol 9 (6): 941-6, 1991.  [PUBMED Abstract]

  116. Thomas DA, Faderl S, O'Brien S, et al.: Chemoimmunotherapy with hyper-CVAD plus rituximab for the treatment of adult Burkitt and Burkitt-type lymphoma or acute lymphoblastic leukemia. Cancer 106 (7): 1569-80, 2006.  [PUBMED Abstract]

  117. Soussain C, Patte C, Ostronoff M, et al.: Small noncleaved cell lymphoma and leukemia in adults. A retrospective study of 65 adults treated with the LMB pediatric protocols. Blood 85 (3): 664-74, 1995.  [PUBMED Abstract]

  118. Magrath I, Adde M, Shad A, et al.: Adults and children with small non-cleaved-cell lymphoma have a similar excellent outcome when treated with the same chemotherapy regimen. J Clin Oncol 14 (3): 925-34, 1996.  [PUBMED Abstract]

  119. Adde M, Shad A, Venzon D, et al.: Additional chemotherapy agents improve treatment outcome for children and adults with advanced B-cell lymphomas. Semin Oncol 25 (2 Suppl 4): 33-9; discussion 45-8, 1998.  [PUBMED Abstract]

  120. Hoelzer D, Ludwig WD, Thiel E, et al.: Improved outcome in adult B-cell acute lymphoblastic leukemia. Blood 87 (2): 495-508, 1996.  [PUBMED Abstract]

  121. Lee EJ, Petroni GR, Schiffer CA, et al.: Brief-duration high-intensity chemotherapy for patients with small noncleaved-cell lymphoma or FAB L3 acute lymphocytic leukemia: results of cancer and leukemia group B study 9251. J Clin Oncol 19 (20): 4014-22, 2001.  [PUBMED Abstract]

  122. Mead GM, Sydes MR, Walewski J, et al.: An international evaluation of CODOX-M and CODOX-M alternating with IVAC in adult Burkitt's lymphoma: results of United Kingdom Lymphoma Group LY06 study. Ann Oncol 13 (8): 1264-74, 2002.  [PUBMED Abstract]

  123. Freedman AS, Takvorian T, Anderson KC, et al.: Autologous bone marrow transplantation in B-cell non-Hodgkin's lymphoma: very low treatment-related mortality in 100 patients in sensitive relapse. J Clin Oncol 8 (5): 784-91, 1990.  [PUBMED Abstract]

  124. Sweetenham JW, Pearce R, Philip T, et al.: High-dose therapy and autologous bone marrow transplantation for intermediate and high grade non-Hodgkin's lymphoma in patients aged 55 years and over: results from the European Group for Bone Marrow Transplantation. The EBMT Lymphoma Working Party. Bone Marrow Transplant 14 (6): 981-7, 1994.  [PUBMED Abstract]

  125. Rizzieri DA, Johnson JL, Niedzwiecki D, et al.: Intensive chemotherapy with and without cranial radiation for Burkitt leukemia and lymphoma: final results of Cancer and Leukemia Group B Study 9251. Cancer 100 (7): 1438-48, 2004.  [PUBMED Abstract]

  126. Morel P, Lepage E, Brice P, et al.: Prognosis and treatment of lymphoblastic lymphoma in adults: a report on 80 patients. J Clin Oncol 10 (7): 1078-85, 1992.  [PUBMED Abstract]

  127. Verdonck LF, Dekker AW, de Gast GC, et al.: Autologous bone marrow transplantation for adult poor-risk lymphoblastic lymphoma in first remission. J Clin Oncol 10 (4): 644-6, 1992.  [PUBMED Abstract]

  128. Thomas DA, O'Brien S, Cortes J, et al.: Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma. Blood 104 (6): 1624-30, 2004.  [PUBMED Abstract]

  129. Sweetenham JW, Santini G, Qian W, et al.: High-dose therapy and autologous stem-cell transplantation versus conventional-dose consolidation/maintenance therapy as postremission therapy for adult patients with lymphoblastic lymphoma: results of a randomized trial of the European Group for Blood and Marrow Transplantation and the United Kingdom Lymphoma Group. J Clin Oncol 19 (11): 2927-36, 2001.  [PUBMED Abstract]

  130. Höllsberg P, Hafler DA: Seminars in medicine of the Beth Israel Hospital, Boston. Pathogenesis of diseases induced by human lymphotropic virus type I infection. N Engl J Med 328 (16): 1173-82, 1993.  [PUBMED Abstract]

  131. Foss FM, Aquino SL, Ferry JA: Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 10-2003. A 72-year-old man with rapidly progressive leukemia, rash, and multiorgan failure. N Engl J Med 348 (13): 1267-75, 2003.  [PUBMED Abstract]

  132. Shimoyama M: Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). Br J Haematol 79 (3): 428-37, 1991.  [PUBMED Abstract]

  133. Takasaki Y, Iwanaga M, Imaizumi Y, et al.: Long-term study of indolent adult T-cell leukemia-lymphoma. Blood 115 (22): 4337-43, 2010.  [PUBMED Abstract]

  134. Yamada Y, Tomonaga M, Fukuda H, et al.: A new G-CSF-supported combination chemotherapy, LSG15, for adult T-cell leukaemia-lymphoma: Japan Clinical Oncology Group Study 9303. Br J Haematol 113 (2): 375-82, 2001.  [PUBMED Abstract]

  135. Fukushima T, Miyazaki Y, Honda S, et al.: Allogeneic hematopoietic stem cell transplantation provides sustained long-term survival for patients with adult T-cell leukemia/lymphoma. Leukemia 19 (5): 829-34, 2005.  [PUBMED Abstract]

  136. Katsuya H, Yamanaka T, Ishitsuka K, et al.: Prognostic index for acute- and lymphoma-type adult T-cell leukemia/lymphoma. J Clin Oncol 30 (14): 1635-40, 2012.  [PUBMED Abstract]

  137. Itonaga H, Tsushima H, Taguchi J, et al.: Treatment of relapsed adult T-cell leukemia/lymphoma after allogeneic hematopoietic stem cell transplantation: the Nagasaki Transplant Group experience. Blood 121 (1): 219-25, 2013.  [PUBMED Abstract]

  138. Ishida T, Hishizawa M, Kato K, et al.: Allogeneic hematopoietic stem cell transplantation for adult T-cell leukemia-lymphoma with special emphasis on preconditioning regimen: a nationwide retrospective study. Blood 120 (8): 1734-41, 2012.  [PUBMED Abstract]

  139. Gill PS, Harrington W Jr, Kaplan MH, et al.: Treatment of adult T-cell leukemia-lymphoma with a combination of interferon alfa and zidovudine. N Engl J Med 332 (26): 1744-8, 1995.  [PUBMED Abstract]

  140. Matutes E, Taylor GP, Cavenagh J, et al.: Interferon alpha and zidovudine therapy in adult T-cell leukaemia lymphoma: response and outcome in 15 patients. Br J Haematol 113 (3): 779-84, 2001.  [PUBMED Abstract]

  141. Hermine O, Allard I, Lévy V, et al.: A prospective phase II clinical trial with the use of zidovudine and interferon-alpha in the acute and lymphoma forms of adult T-cell leukemia/lymphoma. Hematol J 3 (6): 276-82, 2002.  [PUBMED Abstract]

  142. Bazarbachi A, Plumelle Y, Carlos Ramos J, et al.: Meta-analysis on the use of zidovudine and interferon-alfa in adult T-cell leukemia/lymphoma showing improved survival in the leukemic subtypes. J Clin Oncol 28 (27): 4177-83, 2010.  [PUBMED Abstract]

  143. Bazarbachi A, Suarez F, Fields P, et al.: How I treat adult T-cell leukemia/lymphoma. Blood 118 (7): 1736-45, 2011.  [PUBMED Abstract]

  144. Simone CB 2nd, Morris JC, Stewart DM, et al.: Radiation therapy for the management of patients with HTLV-1-associated adult T-cell leukemia/lymphoma. Blood 120 (9): 1816-9, 2012.  [PUBMED Abstract]

  145. Pugh WC: Is the working formulation adequate for the classification of the low grade lymphomas? Leuk Lymphoma 10 (Suppl 1): 1-8, 1993. 

  146. Norton AJ, Matthews J, Pappa V, et al.: Mantle cell lymphoma: natural history defined in a serially biopsied population over a 20-year period. Ann Oncol 6 (3): 249-56, 1995.  [PUBMED Abstract]

  147. Zucca E, Roggero E, Pinotti G, et al.: Patterns of survival in mantle cell lymphoma. Ann Oncol 6 (3): 257-62, 1995.  [PUBMED Abstract]

  148. Campo E, Raffeld M, Jaffe ES: Mantle-cell lymphoma. Semin Hematol 36 (2): 115-27, 1999.  [PUBMED Abstract]

  149. Pérez-Galán P, Dreyling M, Wiestner A: Mantle cell lymphoma: biology, pathogenesis, and the molecular basis of treatment in the genomic era. Blood 117 (1): 26-38, 2011.  [PUBMED Abstract]

  150. Weisenburger DD, Armitage JO: Mantle cell lymphoma-- an entity comes of age. Blood 87 (11): 4483-94, 1996.  [PUBMED Abstract]

  151. Herrmann A, Hoster E, Zwingers T, et al.: Improvement of overall survival in advanced stage mantle cell lymphoma. J Clin Oncol 27 (4): 511-8, 2009.  [PUBMED Abstract]

  152. Fisher RI, Dahlberg S, Nathwani BN, et al.: A clinical analysis of two indolent lymphoma entities: mantle cell lymphoma and marginal zone lymphoma (including the mucosa-associated lymphoid tissue and monocytoid B-cell subcategories): a Southwest Oncology Group study. Blood 85 (4): 1075-82, 1995.  [PUBMED Abstract]

  153. Majlis A, Pugh WC, Rodriguez MA, et al.: Mantle cell lymphoma: correlation of clinical outcome and biologic features with three histologic variants. J Clin Oncol 15 (4): 1664-71, 1997.  [PUBMED Abstract]

  154. Tiemann M, Schrader C, Klapper W, et al.: Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network. Br J Haematol 131 (1): 29-38, 2005.  [PUBMED Abstract]

  155. Lenz G, Dreyling M, Hoster E, et al.: Immunochemotherapy with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone significantly improves response and time to treatment failure, but not long-term outcome in patients with previously untreated mantle cell lymphoma: results of a prospective randomized trial of the German Low Grade Lymphoma Study Group (GLSG). J Clin Oncol 23 (9): 1984-92, 2005.  [PUBMED Abstract]

  156. Romaguera JE, Fayad L, Rodriguez MA, et al.: High rate of durable remissions after treatment of newly diagnosed aggressive mantle-cell lymphoma with rituximab plus hyper-CVAD alternating with rituximab plus high-dose methotrexate and cytarabine. J Clin Oncol 23 (28): 7013-23, 2005.  [PUBMED Abstract]

  157. Witzig TE: Current treatment approaches for mantle-cell lymphoma. J Clin Oncol 23 (26): 6409-14, 2005.  [PUBMED Abstract]

  158. Griffiths R, Mikhael J, Gleeson M, et al.: Addition of rituximab to chemotherapy alone as first-line therapy improves overall survival in elderly patients with mantle cell lymphoma. Blood 118 (18): 4808-16, 2011.  [PUBMED Abstract]

  159. LaCasce AS, Vandergrift JL, Rodriguez MA, et al.: Comparative outcome of initial therapy for younger patients with mantle cell lymphoma: an analysis from the NCCN NHL Database. Blood 119 (9): 2093-9, 2012.  [PUBMED Abstract]

  160. Kluin-Nelemans HC, Hoster E, Hermine O, et al.: Treatment of older patients with mantle-cell lymphoma. N Engl J Med 367 (6): 520-31, 2012.  [PUBMED Abstract]

  161. Martin P, Chadburn A, Christos P, et al.: Outcome of deferred initial therapy in mantle-cell lymphoma. J Clin Oncol 27 (8): 1209-13, 2009.  [PUBMED Abstract]

  162. Khouri IF, Lee MS, Saliba RM, et al.: Nonablative allogeneic stem-cell transplantation for advanced/recurrent mantle-cell lymphoma. J Clin Oncol 21 (23): 4407-12, 2003.  [PUBMED Abstract]

  163. Khouri IF, Romaguera J, Kantarjian H, et al.: Hyper-CVAD and high-dose methotrexate/cytarabine followed by stem-cell transplantation: an active regimen for aggressive mantle-cell lymphoma. J Clin Oncol 16 (12): 3803-9, 1998.  [PUBMED Abstract]

  164. Lefrère F, Delmer A, Suzan F, et al.: Sequential chemotherapy by CHOP and DHAP regimens followed by high-dose therapy with stem cell transplantation induces a high rate of complete response and improves event-free survival in mantle cell lymphoma: a prospective study. Leukemia 16 (4): 587-93, 2002.  [PUBMED Abstract]

  165. Gopal AK, Rajendran JG, Petersdorf SH, et al.: High-dose chemo-radioimmunotherapy with autologous stem cell support for relapsed mantle cell lymphoma. Blood 99 (9): 3158-62, 2002.  [PUBMED Abstract]

  166. Gianni AM, Magni M, Martelli M, et al.: Long-term remission in mantle cell lymphoma following high-dose sequential chemotherapy and in vivo rituximab-purged stem cell autografting (R-HDS regimen). Blood 102 (2): 749-55, 2003.  [PUBMED Abstract]

  167. Forstpointner R, Dreyling M, Repp R, et al.: The addition of rituximab to a combination of fludarabine, cyclophosphamide, mitoxantrone (FCM) significantly increases the response rate and prolongs survival as compared with FCM alone in patients with relapsed and refractory follicular and mantle cell lymphomas: results of a prospective randomized study of the German Low-Grade Lymphoma Study Group. Blood 104 (10): 3064-71, 2004.  [PUBMED Abstract]

  168. Dreyling M, Lenz G, Hoster E, et al.: Early consolidation by myeloablative radiochemotherapy followed by autologous stem cell transplantation in first remission significantly prolongs progression-free survival in mantle-cell lymphoma: results of a prospective randomized trial of the European MCL Network. Blood 105 (7): 2677-84, 2005.  [PUBMED Abstract]

  169. Geisler CH, Kolstad A, Laurell A, et al.: Long-term progression-free survival of mantle cell lymphoma after intensive front-line immunochemotherapy with in vivo-purged stem cell rescue: a nonrandomized phase 2 multicenter study by the Nordic Lymphoma Group. Blood 112 (7): 2687-93, 2008.  [PUBMED Abstract]

  170. Tam CS, Bassett R, Ledesma C, et al.: Mature results of the M. D. Anderson Cancer Center risk-adapted transplantation strategy in mantle cell lymphoma. Blood 113 (18): 4144-52, 2009.  [PUBMED Abstract]

  171. Damon LE, Johnson JL, Niedzwiecki D, et al.: Immunochemotherapy and autologous stem-cell transplantation for untreated patients with mantle-cell lymphoma: CALGB 59909. J Clin Oncol 27 (36): 6101-8, 2009.  [PUBMED Abstract]

  172. Fisher RI, Bernstein SH, Kahl BS, et al.: Multicenter phase II study of bortezomib in patients with relapsed or refractory mantle cell lymphoma. J Clin Oncol 24 (30): 4867-74, 2006.  [PUBMED Abstract]

  173. O'Connor OA, Moskowitz C, Portlock C, et al.: Patients with chemotherapy-refractory mantle cell lymphoma experience high response rates and identical progression-free survivals compared with patients with relapsed disease following treatment with single agent bortezomib: results of a multicentre Phase 2 clinical trial. Br J Haematol 145 (1): 34-9, 2009.  [PUBMED Abstract]

  174. Morrison VA, Dunn DL, Manivel JC, et al.: Clinical characteristics of post-transplant lymphoproliferative disorders. Am J Med 97 (1): 14-24, 1994.  [PUBMED Abstract]

  175. Knowles DM, Cesarman E, Chadburn A, et al.: Correlative morphologic and molecular genetic analysis demonstrates three distinct categories of posttransplantation lymphoproliferative disorders. Blood 85 (2): 552-65, 1995.  [PUBMED Abstract]

  176. Leblond V, Dhedin N, Mamzer Bruneel MF, et al.: Identification of prognostic factors in 61 patients with posttransplantation lymphoproliferative disorders. J Clin Oncol 19 (3): 772-8, 2001.  [PUBMED Abstract]

  177. Ghobrial IM, Habermann TM, Maurer MJ, et al.: Prognostic analysis for survival in adult solid organ transplant recipients with post-transplantation lymphoproliferative disorders. J Clin Oncol 23 (30): 7574-82, 2005.  [PUBMED Abstract]

  178. Armitage JM, Kormos RL, Stuart RS, et al.: Posttransplant lymphoproliferative disease in thoracic organ transplant patients: ten years of cyclosporine-based immunosuppression. J Heart Lung Transplant 10 (6): 877-86; discussion 886-7, 1991 Nov-Dec.  [PUBMED Abstract]

  179. Evens AM, David KA, Helenowski I, et al.: Multicenter analysis of 80 solid organ transplantation recipients with post-transplantation lymphoproliferative disease: outcomes and prognostic factors in the modern era. J Clin Oncol 28 (6): 1038-46, 2010.  [PUBMED Abstract]

  180. Kuehnle I, Huls MH, Liu Z, et al.: CD20 monoclonal antibody (rituximab) for therapy of Epstein-Barr virus lymphoma after hemopoietic stem-cell transplantation. Blood 95 (4): 1502-5, 2000.  [PUBMED Abstract]

  181. Shapiro RS, Chauvenet A, McGuire W, et al.: Treatment of B-cell lymphoproliferative disorders with interferon alfa and intravenous gamma globulin. N Engl J Med 318 (20): 1334, 1988.  [PUBMED Abstract]

  182. Leblond V, Sutton L, Dorent R, et al.: Lymphoproliferative disorders after organ transplantation: a report of 24 cases observed in a single center. J Clin Oncol 13 (4): 961-8, 1995.  [PUBMED Abstract]

  183. Mamzer-Bruneel MF, Lomé C, Morelon E, et al.: Durable remission after aggressive chemotherapy for very late post-kidney transplant lymphoproliferation: A report of 16 cases observed in a single center. J Clin Oncol 18 (21): 3622-32, 2000.  [PUBMED Abstract]

  184. Swinnen LJ: Durable remission after aggressive chemotherapy for post-cardiac transplant lymphoproliferation. Leuk Lymphoma 28 (1-2): 89-101, 1997.  [PUBMED Abstract]

  185. McCarthy M, Ramage J, McNair A, et al.: The clinical diversity and role of chemotherapy in lymphoproliferative disorder in liver transplant recipients. J Hepatol 27 (6): 1015-21, 1997.  [PUBMED Abstract]

  186. Leblond V, Davi F, Charlotte F, et al.: Posttransplant lymphoproliferative disorders not associated with Epstein-Barr virus: a distinct entity? J Clin Oncol 16 (6): 2052-9, 1998.  [PUBMED Abstract]

  187. Senderowicz AM, Vitetta E, Headlee D, et al.: Complete sustained response of a refractory, post-transplantation, large B-cell lymphoma to an anti-CD22 immunotoxin. Ann Intern Med 126 (11): 882-5, 1997.  [PUBMED Abstract]

  188. Haddad E, Paczesny S, Leblond V, et al.: Treatment of B-lymphoproliferative disorder with a monoclonal anti-interleukin-6 antibody in 12 patients: a multicenter phase 1-2 clinical trial. Blood 97 (6): 1590-7, 2001.  [PUBMED Abstract]

  189. Soslow RA, Davis RE, Warnke RA, et al.: True histiocytic lymphoma following therapy for lymphoblastic neoplasms. Blood 87 (12): 5207-12, 1996.  [PUBMED Abstract]

  190. Kamel OW, Gocke CD, Kell DL, et al.: True histiocytic lymphoma: a study of 12 cases based on current definition. Leuk Lymphoma 18 (1-2): 81-6, 1995.  [PUBMED Abstract]

  191. Nador RG, Cesarman E, Chadburn A, et al.: Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood 88 (2): 645-56, 1996.  [PUBMED Abstract]