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Castleman disease

Disease definition

A rare lymphoproliferative disorder characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.

ORPHA:160

Classification level: Disorder
  • Synonym(s):
    • Angiofollicular ganglionic hyperplasia
    • Angiofollicular lymph hyperplasia
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: D47.7
  • ICD-11: 4B2Y
  • OMIM: 148000
  • UMLS: C0017531
  • MeSH: D005871
  • GARD: 12656
  • MedDRA: 10050251

  A summary on this disease is available in Português (2001) Italiano (2006) Deutsch (2014) Français (2014) Español (2023) Nederlands (2023) Greek (2014, pdf)

Detailed information

Guidelines

Disease review articles

  • Review article
  • English (2018) - Hematology Am Soc Hematol Educ Program

Clinical Outcome Assessment (COA)

  • Patient-Centered Outcome Measures (PCOMs)
  • English (2023) - PROQOLIDTM
ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.