Diagnostic delay and histopathological subtyping challenges in idiopathic multicentric Castleman disease, not otherwise specified: a case report of a three-hospital odyssey - Takeaways - MDSpire

Challenges in Diagnosis and Histopathological Classification of Idiopathic Multicentric Castleman Disease: A Case Study Involving Multiple Healthcare Facilities

  • By

  • Fen Zhang

  • Nan Wang

  • Ying-yu Wang

  • Ye Peng

  • Yan-chen Bao

  • Hua-zheng Shi

  • Qian-wen Xiao

  • Xiao-li Zhang

  • Xiao-xi Wang

  • Chun-mei Liao

  • Jing Pan

  • Wei-qiang Teng

  • July 20, 2026

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  • 1

    Castleman disease (CD) is a rare lymphoproliferative disorder with diagnostic challenges due to its heterogeneous nature and nonspecific clinical manifestations.

  • 2

    A 53-year-old male presented with persistent lymphadenopathy and systemic symptoms, leading to a diagnosis of idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS).

  • 3

    Histopathological examination revealed disrupted lymph node architecture and mixed-type Castleman disease, emphasizing the importance of excisional biopsy for accurate diagnosis.

  • 4

    The case highlights the necessity of systematically excluding HHV-8 infection and other mimickers to achieve proper subtyping of iMCD-NOS.

  • 5

    The patient's treatment with siltuximab and prednisone resulted in significant symptom improvement, demonstrating the potential effectiveness of targeted therapy.

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