Evolving idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever/hyperinflammation, renal dysfunction and organomegaly presenting with severe hypoalbuminaemia, serositis and bicytopenia before lymphadenopathy.
John Kachope, Olga Muradov
✦ AI-curated · Sources linked
Case in brief
A man in his early 60s presented with abdominal symptoms, severe hypoalbuminaemia, thrombocytopenia, and marked inflammation, without evident lymphadenopathy. His condition progressed to anasarca, serositis, bicytopenia, and renal dysfunction. After extensive investigations, lymphadenopathy was identified via PET-CT, leading to a diagnosis of idiopathic multicentric Castleman disease with TAFRO features. Treatment with corticosteroids and siltuximab resulted in clinical and biochemical improvement.
What made this case unusual
The case highlights the presentation of TAFRO syndrome with severe hypoalbuminaemia and multisystem involvement prior to the appearance of lymphadenopathy, which is typically a hallmark of Castleman disease.
Diagnostic / clinical pearl
Clinicians should consider idiopathic multicentric Castleman disease in patients presenting with severe hypoalbuminaemia and multisystem symptoms, even in the absence of lymphadenopathy.
Why it matters
Recognizing TAFRO syndrome early can lead to timely diagnosis and management, potentially improving patient outcomes in cases of idiopathic multicentric Castleman disease.