Journal
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
Volume 32, Issue 1, Pages 75-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.hoc.2017.09.007
Keywords
Kaposi sarcoma herpesvirus; Human herpesvirus-8; Multicentric Castleman disease; Human interleukin-6; Viral interleukin 6; Rituximab; Liposomal doxorubicin
Categories
Funding
- Intramural Research Program of the National Institutes of Health National Cancer Institute [ZIA BC 011700]
- National Cancer Institute
- Celgene
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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.
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