Diagnostic and therapeutic challenges in hyaline-vascular idiopathic multicentric Castleman disease: A case report

The diagnosis of idiopathic multicentric Castleman disease typically requires serum interleukin-6 testing-often inaccessible in resource-limited settings. A female patient in her early 40s presented with 6-month progressive generalized pruritus, low-grade fever, fatigue, and painless multiregional lymphadenopathy. Laboratory studies showed systemic inflammation, computed tomography/ultrasound-confirmed bilateral axillary lymphadenopathy, splenomegaly, and serositis. Lymph node biopsy revealed hyaline-vascular features: atrophic germinal centers with a "lollipop-like" vasculature and "onion skin-like" lymphocyte proliferation. Interleukin-6 testing was unavailable. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy (nine cycles) combined with thalidomide (100 mg/day, cycles 6-9) was administered. Significant lymph node regression normalized the levels of inflammatory markers, and partial remission was observed at the 1-year follow-up. Rigorous histopathology combined with dynamic inflammatory markers enables the diagnosis of idiopathic multicentric Castleman disease without interleukin-6 testing. CHOP-thalidomide is an effective alternative therapy in resource-constrained regions.