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Treatment of Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease.


ABSTRACT: 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.

SUBMITTER: Lurain K 

PROVIDER: S-EPMC5726416 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Treatment of Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease.

Lurain Kathryn K   Yarchoan Robert R   Uldrick Thomas S TS  

Hematology/oncology clinics of North America 20180201 1


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  ...[more]

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