Multicentric Castleman Disease Following Kaposi Sarcoma in a Woman Living With HIV: A Case Report

Abstract

Background: Castleman disease comprises a heterogeneous group of lymphoproliferative disorders with shared lymph-node histopathological features. Human herpesvirus 8 (HHV8)-associated multicentric Castleman disease is strongly linked to human immunodeficiency virus (HIV) infection and may occur in patients with current or previous Kaposi sarcoma. We describe biopsy-proven multicentric Castleman disease in a virally suppressed woman living with HIV eight years after remission of Kaposi sarcoma in a resource-limited setting; HHV8 association could not be laboratory-confirmed because HHV8-specific testing was unavailable.

Case presentation: A 48-year-old woman on antiretroviral therapy, with HIV RNA <40 copies/mL and previous Kaposi sarcoma treated with six cycles of liposomal doxorubicin, presented with a one-year history of fatigue, weight loss, night sweats, abdominal distension and left lower-limb oedema. Examination showed pallor, generalised lymphadenopathy, hepatosplenomegaly and firm chronic left-leg oedema. Haemoglobin was 6.7 g/dL. Serum protein electrophoresis showed hyperproteinaemia with a markedly increased gamma fraction. Axillary lymph-node histology demonstrated regressed germinal centres, hyalinised penetrating vessels and concentric mantle-zone lymphocytes, supporting Castleman disease. Cross-sectional imaging and HHV8 LANA-1 immunohistochemistry were unavailable.

Intervention and outcome: The patient was referred for rituximab-based chemoimmunotherapy. The submitted clinical record described the planned regimen as R-EPOCH, but individual drug doses, confirmation of treatment administration and post-treatment outcomes were not available at manuscript preparation.

Conclusion: Multicentric Castleman disease should be considered in people living with HIV who develop persistent constitutional symptoms, generalised lymphadenopathy and organomegaly, including those with remote Kaposi sarcoma and suppressed HIV viraemia. Adequate lymph-node biopsy is central to diagnosis, while HHV8-specific testing should be obtained whenever feasible before assigning an HHV8-associated subtype.