SHM Converge Scientific Abstract ePoster Gallery-Abdominal Distention, Fatigue, and Lower Extremity Edema in a Healthy Young Male: A Case of Hhv8-Negative Multicentric Castleman's Disease

Abdominal Distention, Fatigue, and Lower Extremity Edema in a Healthy Young Male: A Case of Hhv8-Negative Multicentric Castleman's Disease

Back to course

Pdf Summary

This poster describes a 32-year-old previously healthy man who developed abdominal distention, fatigue, night sweats, lower-extremity edema, and lymphadenopathy. Initial workup showed anemia, thrombocytopenia, elevated inflammatory markers (ESR, CRP, ferritin), acute kidney injury, hepatosplenomegaly, pleural effusions, ascites, and diffuse lymphadenopathy. Extensive infectious, rheumatologic, and malignancy evaluations were initially negative, and an early lymph node biopsy showed only reactive hyperplasia.<br /><br />Because suspicion remained high, a PET scan was used to guide a repeat excisional cervical lymph node biopsy. Pathology revealed classic Castleman’s features, including “onion-skinning” of the mantle zone and “lollipop follicles” with vascular proliferation. Based on the clinical picture and biopsy findings, he was diagnosed with HHV-8–negative idiopathic multicentric Castleman disease (iMCD), specifically the TAFRO variant.<br /><br />The poster emphasizes that iMCD is a cytokine-driven disorder, with interleukin-6 (IL-6) thought to play a central role in causing systemic symptoms. Steroids are commonly used first, while refractory cases are often treated with anti-IL-6 therapies such as siltuximab or anti-IL-6 receptor therapy such as tocilizumab. The discussion also notes that renal-limited thrombotic microangiopathy can occur in iMCD, likely related to IL-6–associated prothrombotic effects.<br /><br />Overall, the case highlights the importance of considering Castleman disease in patients with unexplained lymphadenopathy, B symptoms, inflammation, edema, and multisystem involvement, while carefully excluding mimics such as lymphoma, tuberculosis, rheumatoid disease, and connective tissue disorders.

Asset Subtitle

Rebekah L. Roll

Meta Tag

Author List Danielle Wallace, Gregory Matos, Meghan Train, Rebekah L. Roll

Category Clinical Vignettes

Concept Idiopathic Multicentric Castleman Disease

Concept Interleukin-6 Dysregulation

Concept Lymphadenopathy

Concept Interleukin-6Targeted Therapy

Concept HHV-8 Negative Castleman Disease

Distinguished Non-Finalist

Presenter Organization University of Rochester

Presenting Author Rebekah L. Roll

Track Adult

Keywords

Castleman disease

idiopathic multicentric Castleman disease

iMCD

TAFRO syndrome

HHV-8 negative

lymphadenopathy

interleukin-6

thrombotic microangiopathy

PET scan

lymph node biopsy

Idiopathic Multicentric Castleman Disease

Interleukin-6 Dysregulation

Lymphadenopathy

Interleukin-6Targeted Therapy

HHV-8 Negative Castleman Disease