| Systemic mastocytosis in adults: 2012 Update on diagnosis, risk stratification, and management. | |
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MedLine Citation:
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PMID: 22410759 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Animesh Pardanani |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: American journal of hematology Volume: 87 ISSN: 1096-8652 ISO Abbreviation: Am. J. Hematol. Publication Date: 2012 Apr |
Date Detail:
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Created Date: 2012-03-13 Completed Date: 2012-05-29 Revised Date: 2013-05-20 |
Medline Journal Info:
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Nlm Unique ID: 7610369 Medline TA: Am J Hematol Country: United States |
Other Details:
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Languages: eng Pagination: 401-11 Citation Subset: IM |
Copyright Information:
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Copyright © 2012 Wiley Periodicals, Inc. |
Affiliation:
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Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA. pardanani.animesh@mayo.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Cortex Hormones
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therapeutic use Adult Bone Marrow Examination Cell Lineage Cladribine / adverse effects, therapeutic use Clinical Trials as Topic Clone Cells / pathology Disease Management Disease Progression Humans Hydroxyurea / therapeutic use Immunophenotyping Interferon-alpha / therapeutic use Leukemia, Mast-Cell / pathology Mast Cells / chemistry, enzymology, pathology Mastocytosis, Systemic* / classification, diagnosis, epidemiology, genetics, therapy Mutation, Missense Organ Specificity Prognosis Protein Kinase Inhibitors / therapeutic use Proto-Oncogene Proteins c-kit / genetics Risk Assessment Staurosporine / analogs & derivatives, therapeutic use Tryptases / blood |
| Chemical | |
Reg. No./Substance:
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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 2.7.10.1/Proto-Oncogene Proteins c-kit; EC 3.4.21.59/Tryptases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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