HHV 8 ASSOCIATED MULTICENTRIC CASTLEMAN DISEASE IN HIV: DIAGNOSIS MADE ONLY BY EXCISIONAL LYMPH NODE BIOPSY

Case Presentation: A middle-aged man with chronic HIV on integrase inhibitor–based antiretroviral therapy presented with high fevers, malaise, odynophagia, cough, and emesis. Initial studies revealed pancytopenia (WBC 3.7×10^3/µL, hemoglobin 7.6 g/dL, platelets 47×10^3/µL), elevated LDH, mild hyponatremia, and indirect hyperbilirubinemia. CT imaging showed splenomegaly (15–16 cm) with multistation abdominopelvic lymphadenopathy.Bone-marrow biopsy demonstrated mildly hypercellular marrow with focal hemophagocytosis; cytogenetics, flow cytometry, and molecular testing were nondiagnostic. He returned weeks later with recurrent fever and pleuritic chest/flank pain. Repeat CT revealed progressive lymphadenopathy, splenomegaly increasing to ~18 cm, and a new splenic infarct. Percutaneous inguinal lymph-node core biopsy failed to provide a diagnosis.An excisional inguinal lymph-node biopsy was then pursued. Histopathology showed atretic germinal centers with mantle-zone “onion-skinning,” vascular proliferation, polytypic plasmacytosis, and patchy HHV-8 LANA-1 positivity, confirming HHV-8–associated MCD. Broad infectious evaluations, including endemic fungal studies, were negative. With supportive care and continued antiretroviral therapy, the patient’s cytopenias improved, including normalization of platelets. Hematology coordinated outpatient rituximab-based therapy.

Discussion: This case illustrates how HHV-8–associated MCD can closely resemble other systemic inflammatory syndromes, leading to diagnostic uncertainty. Bone-marrow findings may be nonspecific, and core lymph-node biopsies frequently miss the architectural features required for diagnosis. Excisional lymph-node sampling remains the gold standard and should be prioritized when patients with HIV present with unexplained fever, cytopenias, splenomegaly, and generalized lymphadenopathy.

Conclusions: In HIV-positive adults with progressive systemic inflammation and multistation lymphadenopathy, clinicians should maintain early suspicion for HHV-8–associated MCD. When imaging, bone marrow, and core biopsies are inconclusive, prompt excisional lymph-node biopsy is critical to establish the diagnosis and initiate timely rituximab-based therapy. Early identification may substantially reduce morbidity and prevent further complications.