Treatment for Hairy Cell Leukemia
Untreated Hairy Cell Leukemia
Hairy cell leukemia is a highly treatable disease. Since it is easily controlled, many patients have prolonged survival with sequential therapies. The decision to treat is based on cytopenias (especially if symptomatic), increasing splenomegaly, indications that the disease is progressing, or the presence of other, usually infectious complications. It is reasonable to offer no therapy if the patient is asymptomatic and blood counts are maintained in an acceptable range.
Progressive Hairy Cell Leukemia
Standard treatment options:
- Cladribine (2-chlorodeoxyadenosine, 2-CdA) given intravenously by continuous infusion, by daily subcutaneous injections, or by 2-hour infusions daily for 5 to 7 days, results in a complete response rate of 50% to 80% and an overall response rate of 85% to 95%.[1-7] The response rate was lower in 979 patients treated with the Group C mechanism of the National Cancer Institute (i.e., 50% complete remission rate, 37% partial remission rate). Responses are durable with this short course of therapy, and patients who relapse often respond to retreatment with cladribine.[8-10] This drug may cause fever and immunosuppression with documented infection in 33% of treated patients. In a retrospective study of patients with cladribine-associated neutropenic fever, filgrastim (G-CSF) did not demonstrate a decrease in the percentage of febrile patients, number of febrile days, or frequency of admissions for antibiotics. (Refer to the PDQ summary on Fever, Sweats, and Hot Flashes for more information on fever.) A potential increased risk for second malignancies with this agent remains controversial.
- Pentostatin given intravenously every other week for 3 to 6 months produces a 50% to 76% complete response rate and an 80% to 87% overall response rate.[12,13] Complete remissions are of substantial duration. In two trials with 9-year median follow-up, relapse-free survival ranged from 56% to 67%.[14,15] Side effects include fever, immunosuppression, cytopenias, and renal dysfunction. (Refer to the PDQ summary on Fever, Sweats, and Hot Flashes for more information on fever.) A randomized comparison of pentostatin and interferon-alpha demonstrated higher and more durable responses to pentostatin.
- Interferon-alpha given subcutaneously 3 times per week for 1 year yields a 10% complete response rate and an 80% overall response rate. The drug frequently produces an influenza-like syndrome early in the course of treatment. Late effects include depression and lethargy. (Refer to the PDQ summary on Depression for more information on lethargy; refer to the PDQ summary on Fatigue.) Responding patients who relapse usually react positively to retreatment with interferon-alpha. Remission can be prolonged with a low-dose maintenance regimen. A randomized comparison of pentostatin and interferon-alpha demonstrated significantly higher and more durable responses to pentostatin.
- Splenectomy will partially or completely normalize the peripheral blood in the vast majority of patients with hairy cell leukemia. Usually little or no change occurs in the bone marrow after splenectomy, and virtually all patients have progressive disease within 12 to 18 months. Therefore, since a number of more effective alternatives are available, splenectomy is playing a decreasing role in the treatment of this disease.
Ongoing trials are studying combinations of cladribine plus the monoclonal antibody rituximab.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated hairy cell leukemia and progressive hairy cell leukemia, initial treatment. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
- Gidron A, Tallman MS: 2-CdA in the treatment of hairy cell leukemia: a review of long-term follow-up. Leuk Lymphoma 47 (11): 2301-7, 2006. [PUBMED Abstract]
- Hoffman MA, Janson D, Rose E, et al.: Treatment of hairy-cell leukemia with cladribine: response, toxicity, and long-term follow-up. J Clin Oncol 15 (3): 1138-42, 1997. [PUBMED Abstract]
- Cheson BD, Sorensen JM, Vena DA, et al.: Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine via the Group C protocol mechanism of the National Cancer Institute: a report of 979 patients. J Clin Oncol 16 (9): 3007-15, 1998. [PUBMED Abstract]
- Goodman GR, Burian C, Koziol JA, et al.: Extended follow-up of patients with hairy cell leukemia after treatment with cladribine. J Clin Oncol 21 (5): 891-6, 2003. [PUBMED Abstract]
- Robak T, Błasińska-Morawiec M, Krykowski E, et al.: 2-chlorodeoxyadenosine (2-CdA) in 2-hour versus 24-hour intravenous infusion in the treatment of patients with hairy cell leukemia. Leuk Lymphoma 22 (1-2): 107-11, 1996. [PUBMED Abstract]
- Robak T, Jamroziak K, Gora-Tybor J, et al.: Cladribine in a weekly versus daily schedule for untreated active hairy cell leukemia: final report from the Polish Adult Leukemia Group (PALG) of a prospective, randomized, multicenter trial. Blood 109 (9): 3672-5, 2007. [PUBMED Abstract]
- Zenhäusern R, Schmitz SF, Solenthaler M, et al.: Randomized trial of daily versus weekly administration of 2-chlorodeoxyadenosine in patients with hairy cell leukemia: a multicenter phase III trial (SAKK 32/98). Leuk Lymphoma 50 (9): 1501-11, 2009. [PUBMED Abstract]
- Jehn U, Bartl R, Dietzfelbinger H, et al.: An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine. Leukemia 18 (9): 1476-81, 2004. [PUBMED Abstract]
- Chadha P, Rademaker AW, Mendiratta P, et al.: Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood 106 (1): 241-6, 2005. [PUBMED Abstract]
- Else M, Dearden CE, Matutes E, et al.: Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis. Br J Haematol 145 (6): 733-40, 2009. [PUBMED Abstract]
- Saven A, Burian C, Adusumalli J, et al.: Filgrastim for cladribine-induced neutropenic fever in patients with hairy cell leukemia. Blood 93 (8): 2471-7, 1999. [PUBMED Abstract]
- Grever M, Kopecky K, Foucar MK, et al.: Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol 13 (4): 974-82, 1995. [PUBMED Abstract]
- Ribeiro P, Bouaffia F, Peaud PY, et al.: Long term outcome of patients with hairy cell leukemia treated with pentostatin. Cancer 85 (1): 65-71, 1999. [PUBMED Abstract]
- Johnston JB, Eisenhauer E, Wainman N, et al.: Long-term outcome following treatment of hairy cell leukemia with pentostatin (Nipent): a National Cancer Institute of Canada study. Semin Oncol 27 (2 Suppl 5): 32-6, 2000. [PUBMED Abstract]
- Flinn IW, Kopecky KJ, Foucar MK, et al.: Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood 96 (9): 2981-6, 2000. [PUBMED Abstract]
- Golomb HM, Ratain MJ, Fefer A, et al.: Randomized study of the duration of treatment with interferon alfa-2B in patients with hairy cell leukemia. J Natl Cancer Inst 80 (5): 369-73, 1988. [PUBMED Abstract]
- Capnist G, Federico M, Chisesi T, et al.: Long term results of interferon treatment in hairy cell leukemia. Italian Cooperative Group of Hairy Cell Leukemia (ICGHCL). Leuk Lymphoma 14 (5-6): 457-64, 1994. [PUBMED Abstract]
- Golomb HM, Vardiman JW: Response to splenectomy in 65 patients with hairy cell leukemia: an evaluation of spleen weight and bone marrow involvement. Blood 61 (2): 349-52, 1983. [PUBMED Abstract]