HHV-8-associated multicentric Castleman disease with concurrent Kaposi sarcoma

Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder typically manifesting with bulky lymphadenopathy in multiple lymph node stations. We describe an atypical presentation of human herpes virus 8 (HHV8)-associated MCD in a middle-aged man with no significant medical history who presented with 1 month of systemic symptoms. He was found to be HIV-1 positive. A physical examination did not reveal palpable lymphadenopathy. A contrast-enhanced CT scan was notable for hepatosplenomegaly and mildly enlarged scattered lymph nodes in the abdomen and pelvis. A positron emission tomography/CT scan demonstrated hypermetabolic cervical chain lymph nodes. Posterior cervical lymph node pathology showed HHV8-positive MCD with concurrent HIV-associated Kaposi sarcoma. The patient was treated with rituximab and liposomal doxorubicin without response. We emphasise the lack of the hallmark of bulky lymphadenopathy in this patient, and the importance of a timely pathological diagnosis in MCD.