Document Detail

Systemic mastocytosis in adults: 2012 Update on diagnosis, risk stratification, and management.
MedLine Citation:
PMID:  22410759     Owner:  NLM     Status:  MEDLINE    
DISEASE OVERVIEW: Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs.
DIAGNOSIS: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V.
RISK STRATIFICATION: The prognostic relevance of the 2008 World Health Organization (WHO) classification of SM has recently been confirmed. Classification of SM patients into indolent (SM), aggressive SM (ASM), SM associated with a clonal non-MC lineage disease (SM-AHNMD) and mast cell leukemia (MCL) subgroups is a useful first step in establishing prognosis.
MANAGEMENT: SM treatment is generally palliative. ISM patients have a normal life expectancy and receive symptom-directed therapy; infrequently, cytoreductive therapy may be indicated for refractory symptoms. ASM patients have disease-related organ dysfunction; interferon-α (±corticosteroids) can control dermatological, hematological, gastrointestinal, skeletal, and mediator-release symptoms, but is hampered by poor tolerability. Similarly, cladribine has broad therapeutic activity, with particular utility when rapid MC debulking is indicated; the main toxicity is myelosuppression. Imatinib has a therapeutic role in the presence of an imatinib-sensitive KIT mutation or in KITD816-unmutated patients. Treatment of SM-AHNMD is governed primarily by the non-MC neoplasm; hydroxyurea has modest utility in this setting.
INVESTIGATIONAL DRUGS: Dasatinib's in vitro anti-KITD816V activity has not translated into significant therapeutic activity in most SM patients. In contrast, preliminary data suggest that Midostaurin may produce significant decreases in MC burden in some patients.
Animesh Pardanani
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  American journal of hematology     Volume:  87     ISSN:  1096-8652     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-03-13     Completed Date:  2012-05-29     Revised Date:  2013-11-06    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  401-11     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Wiley Periodicals, Inc.
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
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MeSH Terms
Adrenal Cortex Hormones / therapeutic use
Bone Marrow Examination
Cell Lineage
Cladribine / adverse effects,  therapeutic use
Clinical Trials as Topic
Clone Cells / pathology
Disease Management
Disease Progression
Hydroxyurea / therapeutic use
Interferon-alpha / therapeutic use
Leukemia, Mast-Cell / pathology
Mast Cells / chemistry,  enzymology,  pathology
Mastocytosis, Systemic* / classification,  diagnosis,  epidemiology,  genetics,  therapy
Mutation, Missense
Organ Specificity
Protein Kinase Inhibitors / therapeutic use
Proto-Oncogene Proteins c-kit / genetics
Risk Assessment
Staurosporine / analogs & derivatives,  therapeutic use
Tryptases / blood
Reg. No./Substance:
0/Adrenal Cortex Hormones; 0/Interferon-alpha; 0/Protein Kinase Inhibitors; 120685-11-2/4'-N-benzoylstaurosporine; 127-07-1/Hydroxyurea; 4291-63-8/Cladribine; 62996-74-1/Staurosporine; EC Proteins c-kit; EC

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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