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Smouldering systemic mastocytosis presenting with cryptic life-threatening crises: case report and literature review.
MedLine Citation:
PMID:  22408224     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Systemic mastocytosis (SM) is a rare, clonal disorder of the mast cell (MC) and its precursor cells. It is characterized by proliferation and accumulation of MCs within various organs, most commonly the skin. The clinical course is variable with indolent or smouldering and aggressive forms being described. We report the case of a middle-aged male patient with smouldering SM presenting with atypical recurrent life-threatening crises. The patient reported a 19-year history of chronic symptoms. The patient had four inpatient stays due to atypical life-threatening crises, during which he has shown end organ damage (cardiac and renal). With each crisis the patient reported acute symptoms. The management of each of these episodes was complex and made more challenging by the patient's longstanding history of hypertension and ischaemic heart disease. In short, SM can present with unexplained life-threatening crises which can be confused for an infectious disease being acute in nature.
Authors:
M Shah; T L Holyoake; B Jackson; M Drummond
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Scottish medical journal     Volume:  57     ISSN:  0036-9330     ISO Abbreviation:  Scott Med J     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-03-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2983335R     Medline TA:  Scott Med J     Country:  Scotland    
Other Details:
Languages:  eng     Pagination:  i-60     Citation Subset:  IM    
Affiliation:
Medical College, CMH Lahore Medical College, University of Health Sciences, Abdur Rahman Road, Lahore Cantt, Pakistan.
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