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Unexplained Anasarca, Cytopenias, and Renal Failur ...
Unexplained Anasarca, Cytopenias, and Renal Failure: Revisiting Early Clues to Diagnose a Castleman Variant
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Pdf Summary
This case describes a 38-year-old unhoused man with progressive anasarca, rash, cytopenias, acute kidney injury, and later dialysis-dependent renal failure, massive ascites, and bacteremia. Because his care had been fragmented across multiple states, the initial diagnostic picture was unclear and broad differentials included infection, autoimmune disease, cirrhosis, and systemic inflammatory syndromes.<br /><br />Key findings helped shift suspicion away from isolated organ disease: kidney biopsy showed a membranoproliferative pattern of glomerular injury, suggesting immune-mediated systemic disease; ascitic studies showed a high SAAG with elevated protein, and liver imaging/biopsy demonstrated portal hypertension without cirrhosis, consistent with non-cirrhotic portal hypertension. These clues pointed toward a multisystem inflammatory disorder rather than simple liver or kidney pathology.<br /><br />After extensive evaluation and involvement of more than seven subspecialties, hospitalists advocated for excisional lymph node biopsy. Pathology confirmed idiopathic multicentric Castleman disease (iMCD) with TAFRO features. This syndrome is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly, and can present with profound diagnostic uncertainty and multiorgan failure.<br /><br />Treatment with IL-6 blockade and rituximab led to stabilization and improvement in systemic inflammation and organ dysfunction. The case highlights several teaching points: cytopenias with systemic inflammation should prompt evaluation for hematologic and inflammatory causes; membranoproliferative glomerulonephritis is a pattern, not a diagnosis; and portal hypertension without cirrhosis should raise concern for vascular or inflammatory etiologies. Most importantly, it emphasizes the hospitalist’s role in coordinating complex consultative care, maintaining diagnostic momentum, and ensuring continuity for vulnerable patients.
Asset Subtitle
Jack B. Ding
Meta Tag
Author List
Jack B. Ding, Lonnie Thach
Category
Clinical Vignettes
Concept
Idiopathic Multicentric Castleman Disease
Concept
TAFRO Syndrome
Concept
Anasarca
Concept
Cytopenia
Concept
Renal Failure
Distinguished
Non-Finalist
Presenter Organization
Georgetown University and Medstar Washington Hospital Center
Presenting Author
Jack B. Ding
Track
Adult
Keywords
idiopathic multicentric Castleman disease
TAFRO syndrome
anasarca
membranoproliferative glomerulonephritis
non-cirrhotic portal hypertension
cytopenias
acute kidney injury
dialysis-dependent renal failure
IL-6 blockade
rituximab
Idiopathic Multicentric Castleman Disease
TAFRO Syndrome
Anasarca
Cytopenia
Renal Failure
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