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Castleman Disease

Wei Wang1, L Jeffrey Medeiros1

  • 1Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.

Surgical Pathology Clinics
|July 30, 2019
PubMed

Insights

Castleman disease (CD) is a diverse group of disorders. Unicentric CD is often cured by surgery, while multicentric CD requires therapy but lacks a standard treatment approach.

Area of Science:

  • Oncology
  • Hematology
  • Pathology

Background:

  • Castleman disease (CD) presents as either unicentric or multicentric.
  • Pathologically, CD is classified into hyaline-vascular and plasma cell variants.

Purpose of the Study:

  • To summarize the clinical and pathological heterogeneity of Castleman disease.
  • To highlight the differing therapeutic approaches for unicentric and multicentric Castleman disease.

Main Methods:

  • Review of existing literature on Castleman disease classification and treatment.
  • Analysis of pathological and clinical subtypes of CD.

Main Results:

  • Unicentric Castleman disease (UCD), predominantly the hyaline-vascular type, is common and often curable with surgical excision.
  • Multicentric Castleman disease (MCD) includes HHV8-associated, idiopathic, and POEMS syndrome-associated subtypes, necessitating therapy but lacking a consensus treatment strategy.

Conclusions:

  • Castleman disease is a heterogeneous entity requiring distinct therapeutic strategies based on subtype.
  • Further research is needed to establish consensus treatment guidelines for multicentric Castleman disease.

Keywords:
Castleman diseaseHuman herpes virus type 8Lymphoproliferative disorderMulticentric Castleman diseaseUnicentric hyaline-vascular Castleman disease

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