An Unusual Presentation of Idiopathic Multicentric Castleman Disease: The Great Masquerader

  • Sujaya Mazumder Dept. of Pathology Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai-400094, India.
  • Raji Tejas Naidu Dept. of Pathology Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai-400094, India.
  • Susan Cherian Dept. of Pathology Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai-400094, India.
  • Sruthi Mayura Dept. of Pathology Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai-400094, India.
  • Uma Pankaj Chaturvedi Dept. of Pathology Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai-400094, India.
DOI: https://doi.org/10.21276/apalm.2844
Keywords: Multicentric, idiopathic, Castleman disease, plasma cells

Abstract

Castleman disease is an uncommon, non-clonal, lymphoproliferative disorder characterized by lymphadenopathy and symptoms related to hypercytokinemia. Clinically it is classified as unicentric and multicentric disease. Multicentric disease is further subclassified as HHV- 8 associated disease and idiopathic disease, which is the rarest subtype. The incidence of idiopathic disease is estimated to be 5 per million person years. The diagnosis of Idiopathic Multicentric Castleman disease is complicated by an array of clinical mimics and non-specific symptoms. We report a rare case of Idiopathic Multicentric Castleman disease in a young female where a detailed pathological work up helped to secure the diagnosis and exclude its mimics.

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Published
2020-09-25
Issue
Vol 7 No 9 (2020)
Section
Case Report

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