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Classification of mastocytosis variants

Classification of mastocytosis variants
Cutaneous mastocytosis (CM)
Urticaria pigmentosa/maculopapular cutaneous mastocytosis
Diffuse cutaneous mastocytosis
Mastocytoma of skin
Systemic mastocytosis (SM)
Indolent systemic mastocytosis* (including the bone marrow mastocytosis subtype)
Smoldering systemic mastocytosis*
Systemic mastocytosis with an associated hematologic neoplasm
Aggressive systemic mastocytosis*
Mast cell leukemia
Mast cell sarcoma
* The complete diagnosis of these variants requires information regarding B and C findings, all of which may not be available at the time of initial tissue diagnosis. Refer to UpToDate content on determining the subtype of systemic mastocytosis for a discussion of B and C findings.
¶ This variant is equivalent to the previously described entity "systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease," and the terms can be used synonymously.
Reprinted with permission from: Horny HP, Metcalfe DD, Akin C, et al. Mastocytosis. In: WHO classification of tumours of haematopoietic and lymphoid tissues, revised 4th edition, Swerdlow SH, Campo E, Harris NL, et al (Eds), IARC, Copyright © 2017.
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