Treatment of Kaposi Sarcoma Herpesvirus–Associated Multicentric Castleman Disease

Publication date: February 2018
Source:Hematology/Oncology Clinics of North America, Volume 32, Issue 1
Author(s): Kathryn Lurain, Robert Yarchoan, Thomas S. Uldrick

Teaser

Kaposi sarcoma herpesvirus (KSHV)-associated multicentric Castleman disease (MCD) is a rare, polyclonal lymphoproliferative disorder characterized by flares of inflammatory symptoms, edema, cytopenias, lymphadenopathy, and splenomegaly. Diagnosis requires a lymph node biopsy. Pathogenesis is related to dysregulated inflammatory cytokines, including human and viral interleukin-6. Rituximab alone or in combination with chemotherapy, such as liposomal doxorubicin, has led to an overall survival of over 90% at 5 years. Experimental approaches to treatment include virus activated cytotoxic therapy with high-dose zidovudine and valganciclovir and targeting human interleukin-6 activity. Despite successful treatment of KSHV-MCD, patients remain at high risk for developing non-Hodgkin lymphomas.


http://ift.tt/2AXFQ1v