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R Bertinchamp1, L Terriou2

  • 1Service de médecine interne et immunologie clinique, CHU Antoine-Béclère, Clamart, France.

La Revue De Medecine Interne
|January 19, 2023
PubMed
Summary
This summary is machine-generated.

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Castleman disease is a rare disorder involving lymph node enlargement. New diagnostic criteria for idiopathic multicentric Castleman disease (iMCD) have been established by experts, requiring specific biopsy findings and ruling out other conditions.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Context:

  • Castleman disease (CD) encompasses rare lymphoproliferative disorders.
  • Two primary subtypes exist: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).
  • MCD further divides into HHV-8 positive MCD and idiopathic MCD (iMCD).

Purpose:

  • To establish diagnostic criteria for idiopathic multicentric Castleman disease (iMCD).
  • To provide a consensus framework for diagnosing iMCD in pediatric and adult patients.

Summary:

  • Castleman disease presents with lymphadenopathy and diverse clinical/laboratory findings.
  • Proposed iMCD criteria mandate characteristic histopathology, multi-regional lymph node enlargement, clinical/lab abnormalities, and exclusion of mimic disorders.
Keywords:
Castleman DiseaseClassificationCritères diagnostiquesDiagnostic criteriaEpidemiologyMaladies de CastlemanÉpidémiologie

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  • Incidence estimates in the US suggest CD affects 6500-7700 individuals annually.

    • Impact:

    • Facilitates accurate diagnosis of iMCD, improving patient management.
    • Aids in differentiating iMCD from other conditions with similar presentations.
    • Supports further research into the pathophysiology and treatment of Castleman disease subtypes.