Idiopathic multicentric Castleman disease in an HIV-positive child.
Noelle Pretorius, Anne-Cecilia van Marle, Christie Esterhuysen et al.
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Case in brief
A preadolescent girl with HIV presented with fever and unilateral facial and neck swelling. Examination showed generalised lymphadenopathy, hepatosplenomegaly, hypertension and haematuria, initially suggesting poststreptococcal glomerulonephritis. With no improvement on supportive treatment, she underwent further evaluation. Her blood smear showed marked agglutination that improved with warming; serum electrophoresis found a monoclonal protein, while bone marrow plasmacytosis was 30%, polyclonal and human herpesvirus 8 negative. Lymph node biopsy established plasmacytic idiopathic multicentric Castleman disease. The atypical presentation delayed diagnosis and led to unnecessary invasive investigations.
What made this case unusual
The combination of a monoclonal serum protein with polyclonal bone marrow plasmacytosis, alongside cold-responsive blood agglutination, complicated the diagnostic picture in this child.
Diagnostic / clinical pearl
In a child with HIV and generalised lymphadenopathy, hepatosplenomegaly and systemic findings, persistent or atypical features should prompt consideration of multicentric Castleman disease; lymph node biopsy established the diagnosis in this case.