Evolving idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever/hyperinflammation, renal dysfunction and organomegaly presenting with severe hypoalbuminaemia, serositis and bicytopenia before lymphadenopathy.

چکیده اصلی

A man in his early 60s presented initially with abdominal symptoms, nutritional deficiencies, severe hypoalbuminaemia, thrombocytopenia and marked inflammation, without clinically apparent lymphadenopathy. Over subsequent hospitalisations, he developed progressive anasarca, pleural and peritoneal serositis, bicytopenia and renal dysfunction. Cardiac, renal, gastrointestinal, infectious, neoplastic and autoimmune explanations were investigated but did not account for the evolving multisystem picture. Lymphadenopathy became evident only later on positron emission tomography-CT, providing the diagnostic pivot. Lymph node assessment, persistent hyperinflammation and the evolving clinical pattern supported idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever/hyperinflammation, renal dysfunction and organomegaly features (iMCD-TAFRO). Treatment with corticosteroids and siltuximab was followed by clinical and biochemical improvement. This case illustrates how TAFRO syndrome may become recognisable before lymphadenopathy makes Castleman disease an obvious consideration.

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