| Systemic mastocytosis: bone marrow pathology, classification, and current therapies. | |
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MedLine Citation:
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PMID: 15995324 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Mast cell disease (MCD) is characterized by the abnormal growth and accumulation of neoplastic mast cells (MC) in one or more organs. The diagnosis of systemic MCD is most commonly established by a thorough histological and immunohistochemical examination of a bone marrow (BM) trephine specimen. In cases with pathognomonic perivascular and -trabecular aggregates of morphologically atypical MC and significant BM involvement, the diagnosis may be relatively straightforward. In contrast, when a sparse, loose pattern of MC infiltration predominates, or when MCs are obscured by an associated non-MC hematological neoplasm, a high index of suspicion and use of adjunctive tests, including special stains, such as tryptase and CD25, may be necessary to reach a diagnosis. The updated classification for MCD clarifies the clinical and pathological criteria for categorizing patients into relatively discrete subgroups. Some cases, however, such those with Fip1-like-1-platelet-derived growth factor receptor alpha (FIP1L1-PDGFRA)(+) clonal eosinophilia associated with elevated serum tryptase levels, with features that overlap MCD and chronic eosinophilic leukemia, may not be easy to categorize on the basis of this classification. There is no standard therapy for MCD and treatment has to be tailored to the needs of the individual patient. MC-cytoreductive therapies, such as interferon-alpha and chemotherapy, are generally reserved for patients with progressive disease and organopathy. A subset of MCD patients with associated eosinophilia who carry the FIP1L1-PDGFRA oncogene will achieve complete clinical, histological, and molecular remissions with imatinib mesylate therapy, in contrast to those with c-kit D816V mutations. The BM pathology, consensus classification, and current therapies for MCD are further discussed in this article. |
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Authors:
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A Pardanani |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Acta haematologica Volume: 114 ISSN: 0001-5792 ISO Abbreviation: Acta Haematol. Publication Date: 2005 |
Date Detail:
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Created Date: 2005-07-04 Completed Date: 2005-08-04 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 0141053 Medline TA: Acta Haematol Country: Switzerland |
Other Details:
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Languages: eng Pagination: 41-51 Citation Subset: IM |
Copyright Information:
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Copyright (c) 2005 S. Karger AG, Basel. |
Affiliation:
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Divisions of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Pardanani.animesh@mayo.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Antineoplastic Agents
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therapeutic use Bone Marrow / pathology* Humans Interferon-alpha / therapeutic use Mastocytosis, Systemic / classification*, pathology*, therapy* Oncogene Proteins, Fusion Piperazines / therapeutic use Pyrimidines / therapeutic use Receptor, Platelet-Derived Growth Factor alpha / analysis Receptors, Interleukin-2 / analysis Serine Endopeptidases / analysis Tryptases mRNA Cleavage and Polyadenylation Factors / analysis |
| Chemical | |
Reg. No./Substance:
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0/Antineoplastic Agents; 0/FIP1L1-PDGFRA fusion protein, human; 0/Interferon-alpha; 0/Oncogene Proteins, Fusion; 0/Piperazines; 0/Pyrimidines; 0/Receptors, Interleukin-2; 0/mRNA Cleavage and Polyadenylation Factors; 152459-95-5/imatinib; EC 2.7.10.1/Receptor, Platelet-Derived Growth Factor alpha; EC 3.4.21.-/Serine Endopeptidases; EC 3.4.21.59/Tryptases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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