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The clinical and pathological panoply of systemic mastocytosis
Author(s) -
Radia Deepti H.,
Green Anna,
Oni Clare,
Moonim Mufaddal
Publication year - 2020
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.16288
Subject(s) - systemic mastocytosis , hepatosplenomegaly , medicine , pathological , disease , chronic myelomonocytic leukemia , myeloid , systemic disease , pathology , immunology , dermatology , bone marrow , myelodysplastic syndromes
Summary Mastocytosis is a rare disease with varied presentation, myriad symptomatology and variable prognosis. Most patients present with cutaneous disease and mediator‐related symptomatology with a small subset having systemic disease (systemic mastocytosis, SM). A subset of the latter develops synchronous or metachronous haematologic neoplasms (SM‐AHN), most commonly chronic myelomonocytic leukaemia (CMML). Advanced systemic mastocytosis (ASM) is seen in a relatively small number of patients and is usually associated with organ dysfunction, and may present with hepatosplenomegaly, lymphadenopathy and ascites with progression to leukaemic transformation (mast cell leukaemia/acute myeloid leukaemia) occurring in a few patients. This paper discusses the clinical and pathologic features of the entire spectrum of SM in adults.