Abstract:
: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.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Radia DH,Green A,Oni C,Moonim Mdoi
10.1111/bjh.16288subject
Has Abstractpub_date
2020-03-01 00:00:00pages
623-640issue
5eissn
0007-1048issn
1365-2141journal_volume
188pub_type
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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pub_type: 杂志文章
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章
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journal_title:British journal of haematology
pub_type: 杂志文章
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journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究,随机对照试验
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