Diagnostic delay and histopathological subtyping challenges in idiopathic multicentric Castleman disease, not otherwise specified: a case report of a three-hospital odyssey - Scorecard - 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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Clinical Scorecard: Challenges in Diagnosis and Histopathological Classification of Idiopathic Multicentric Castleman Disease: A Case Study Involving Multiple Healthcare Facilities

At a Glance

CategoryDetail
ConditionIdiopathic Multicentric Castleman Disease (iMCD-NOS)
Key MechanismsLymphoproliferative disorder with systemic inflammatory symptoms and elevated inflammatory markers.
Target PopulationAdults with persistent lymphadenopathy and systemic symptoms.
Care SettingMultidisciplinary healthcare facilities.

Key Highlights

  • iMCD-NOS is often misdiagnosed due to overlapping features with other conditions.
  • Excisional biopsy is preferred over core needle biopsy for accurate diagnosis.
  • Systematic exclusion of HHV-8 infection and other mimickers is essential.
  • Elevated inflammatory markers and systemic symptoms are key diagnostic indicators.
  • Multicentric lymphadenopathy requires careful clinical evaluation.

Guideline-Based Recommendations

Diagnosis

  • Consider Castleman disease in cases of unexplained lymphadenopathy with systemic symptoms.

Management

  • Treatment with siltuximab plus prednisone has shown improvement in symptoms.

Monitoring & Follow-up

  • Regular follow-up for symptom resolution and recurrence of lymphadenopathy.

Risks

  • Misdiagnosis can lead to unnecessary procedures and delayed treatment.

Patient & Prescribing Data

Adults presenting with persistent lymphadenopathy and systemic inflammatory symptoms.

Combination therapy with siltuximab and prednisone is effective.

Clinical Best Practices

  • Utilize excisional biopsy for definitive diagnosis of iMCD-NOS.
  • Engage in multidisciplinary collaboration for complex cases.
  • Maintain high suspicion for iMCD-NOS in patients with systemic inflammatory symptoms.

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