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Multicentric Castleman disease: Where are we now?

Hao-Wei Wang1, Stefania Pittaluga1, Elaine S Jaffe1

  • 1Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.

Seminars in Diagnostic Pathology
|June 15, 2016
PubMed
Summary

Multicentric Castleman disease (MCD) is a complex disorder involving abnormal cytokine activity leading to inflammation and enlarged lymph nodes. Understanding its subtypes, including KSHV/HHV8-associated and idiopathic forms, is crucial for diagnosis and treatment.

Keywords:
Acquired immune deficiency syndromeCastleman diseaseHuman herpesvirus type-8Interleukin-6Kaposi sarcoma-associated herpesvirusTAFRO syndrome

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Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Multicentric Castleman disease (MCD) presents with diverse clinicopathological features.
  • A central mechanism is dysregulated cytokine activity, causing systemic inflammation and lymphadenopathy.
  • Histological findings in MCD overlap with unicentric Castleman disease variants.

Purpose of the Study:

  • To review evolving concepts and definitions of MCD.
  • To summarize current knowledge on MCD histopathology and pathogenesis.
  • To differentiate KSHV/HHV8-associated MCD from idiopathic MCD.

Main Methods:

  • Literature review of Castleman disease concepts.
  • Analysis of pathogenetic mechanisms in MCD.
  • Comparison of histological findings across MCD subtypes.

Main Results:

  • MCD pathogenesis involves hypercytokinemia, often driven by Kaposi sarcoma-associated herpesvirus/human herpesvirus-8 (KSHV/HHV8) or other mechanisms in idiopathic MCD.
  • KSHV/HHV8-associated MCD can lead to lymphoproliferations and lymphoma risk.
  • Idiopathic MCD presents with varied clinical and pathological features due to diverse hypercytokinemia causes.

Conclusions:

  • MCD is a spectrum of diseases unified by cytokine dysregulation.
  • Distinguishing KSHV/HHV8-positive from idiopathic MCD is important for understanding prognosis and treatment.
  • Further research into the diverse pathogenetic pathways of idiopathic MCD is warranted.