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multicentric form of Castleman disease

Wednesday 6 January 2010


- Nearly always plasma cell type Castleman disease

  • Presents with generalized lymphadenopathy
  • May involve spleen

- Clinical and laboratory features similar to those of angioimmunoblastic lymphadenopathy

- May be associated with:

  • POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes
  • include distinctive vascular lesion ‘glomeruloid hemangioma’
  • amyloid deposits
  • Kaposi sarcoma
  • large cell lymphoma of immunoblastic type

- Tends to persist months or years
- Sometimes renal or pulmonary complications


Etiology unknown but two main hypotheses (not mutually exclusive):

- abnormal immune response
- viral infection

  • definite link between HHV-8 and a subset of multicentric Castleman’s disease characterized by dissolution of lymphoid follicles
  • HHV-8 is also linked to Kaposi sarcoma and primary effusion lymphoma
  • It is hypothesized that HHV-8 induces changes of Castleman disease through interleukin-6 production.

- Some regard it as lymphoproliferative rather than reactive/inflammatory.
- Evidence of clonal rearrangement for immunoglobulin and T-cell receptor genes together with copies of EBV genome
- may evolve into clonal lymphoproliferation

See also

- Castleman disease

  • solitary form of Castleman disease


- Multicentric Castleman disease in an HHV-8-infected child born to consanguineous parents. Leroy S, Moshous D, Cassar O, Reguerre Y, Byun M, Pedergnana V, Canioni D, Gessain A, Oksenhendler E, Fieschi C, Mahlaoui N, Rivière JP, Herbigneaux RM, Muszlak M, Arnaud JP, Fischer A, Picard C, Blanche S, Plancoulaine S, Casanova JL. Pediatrics. 2012 Jan;129(1):e199-203. PMID: 22157133