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Tuesday 16 May 2006


2016 WHO classification

- cutaneous mastocytosis / CM

  • maculopapular cutaneous mastocytosis / MPCM = urticaria pigmentosa / UP
  • diffuse cutaneous mastocytosis / DCM
  • mastocytoma of the skin / cutaneous mastocytoma

- systemic mastocytosis / SM

  • indolent systemic mastocytosis
  • smoldering systemic mastocytosis
  • SM with associated hematologic neoplasm / SM-AHN / SH-AHNMD
  • aggressive systemic mastocytosis
  • mast cell leukemia / MCL

- mast cell sarcoma / MCS


- localized mastocytosis

  • solitary mastocytoma
  • cutaneous mastocytosis
    • diffuse cutaneous mastocytosis
    • solitary cutaneous mastocytosis
    • pigmentaria urticaria

- systemic mastocytosis


See also

- mastocytes


- Mastocytosis: pathology, genetics, and current options for therapy.
Valent P, Akin C, Sperr WR, Mayerhofer M, Födinger M, Fritsche-Polanz R, Sotlar K, Escribano L, Arock M, Horny HP, Metcalfe DD. Leuk Lymphoma. 2005 Jan;46(1):35-48. PMID: 15621779

- Mastocytosis. Metcalfe DD. Novartis Found Symp. 2005;271:232-42; discussion 242-9. PMID: 16605139

- Mastocytosis: state of the art. Horny HP, Sotlar K, Valent P. Pathobiology. 2007;74(2):121-32. PMID: 17587883

- Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. Valent P, Sperr WR, Schwartz LB, Horny HP. J Allergy Clin Immunol. 2004 Jul;114(1):3-11; quiz 12. PMID: 15241337

- Hartmann K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am. 2000 Jun;14(3):625-40. PMID: 10909043

- Mast cell proliferative disorders: current view on variants recognized by the World Health Organization. Valent P, Akin C, Sperr WR, Horny HP, Metcalfe DD. Hematol Oncol Clin North Am. 2003 Oct;17(5):1227-41. PMID: 14560784

- Diagnostic criteria and classification of mastocytosis: a consensus proposal. Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nuñez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM. Leuk Res. 2001 Jul;25(7):603-25. PMID: 11377686