HHV8-associated multicentric Castleman disease with Kaposi sarcoma in an immunocompetent patient: a case report and review of the literature.
K Hoerschgen, W Zhang, A Rappolt et al.
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Case in brief
A 57-year-old man presented with five months of generalized lymphadenopathy, weakness, cough, fever, and weight loss. HIV, CMV, EBV, and tuberculosis tests were negative. Examination of a lymph node showed both Kaposi sarcoma and HHV8-associated multicentric Castleman disease. Sequencing of the two lesions found no driver mutations but identified shared nonsynonymous polymorphisms in eight genes. Despite initial treatment with rituximab and doxorubicin, he died from complications of his illness.
What made this case unusual
Both lesions were present in the same lymph node in an HIV-negative, immunocompetent patient, without mucocutaneous manifestations. The report also profiled the concurrent lesions separately by targeted sequencing.
Diagnostic / clinical pearl
In an HIV-negative patient with generalized lymphadenopathy and systemic symptoms, consider HHV8-associated multicentric Castleman disease even without mucocutaneous signs; lymph-node histology may reveal concurrent Kaposi sarcoma.