mastocytosis

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

Types

 localized mastocytosis

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

 systemic mastocytosis

• SH-AHNMD

See also

 mastocytes

References

 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