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Treatment for Castleman Disease

Name of the Trial

Pilot Study of High-Dose Zidovudine and Valganciclovir With or Without Bortezomib or EPOCH-R (Comprising Etoposide, Doxorubicin, Vincristine, Prednisone, Cyclophosphamide, and Rituximab) or Observation or HAART Only in Patients With Multicentric Castleman Disease Associated With Kaposi Sarcoma-Associated Herpesvirus (NCI-04-C-0275). See the protocol summary.

Principal Investigator

Dr. Richard Little, NCI Center for Cancer Research.

Why Is This Trial Important?

Dr. Richard Little
Dr. Richard Little
Principal Investigator

Multicentric Castleman disease (MCD) is a rare disorder that causes numerous problems, including fatigue, fever, anemia, and tumor-like growths in multiple lymph nodes. Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8, is found in about 50% of MCD cases not associated with HIV, and in nearly 100 percent of HIV-associated MCD. Some patients may also have Kaposi sarcoma.

In this trial, symptomatic patients will be treated with high-dose zidovudine (HDAZT) and valganciclovir. These antiviral drugs are converted into toxic compounds by KSHV-encoded proteins. These toxic compounds may lead to specific killing of KSHV-infected cells (MCD tumor cells). Patients who do not respond to this treatment will also receive the drug bortezomib to see if it can increase the ability of KSHV to activate HDAZT and valganciclovir and increase tumor cell death.

Patients with no symptoms will be monitored without therapy for MCD. HIV-infected patients will receive treatment for HIV, called highly active antiretroviral therapy or HAART, if appropriate. Patients who develop life-threatening disease will be treated with conventional chemotherapy (EPOCH-R) to try to bring their disease and symptoms into remission.

"Laboratory research indicates that certain KSHV genes can activate HDAZT and valganciclovir to kill tumor cells," said Dr. Little. "If this approach works in patients with MCD, it may provide the basis for exploring similar strategies for other viral-associated tumors."

Who Can Join This Trial?

Researchers seek to enroll up to 30 patients aged 12 or over with KSHV-associated MCD. See the list of eligibility criteria.

Where Is This Trial Taking Place?

This study is taking place at the NIH Clinical Center in Bethesda, Md.

Contact Information

For more information, call the NCI Clinical Studies Support Center (CSSC) at 1-888-NCI-1937. The call is toll free and confidential.

  • Posted: December 13, 2005