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    ABSTRACT: Systemic mastocytosis is a heterogeneous disorder characterized by diverse signs and symptoms. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in our molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations. Conventional management and promising novel agents are summarized in this review.
    Immunology and Allergy Clinics of North America 09/2006; 26(3):549-73. DOI:10.1016/j.iac.2006.05.009 · 1.82 Impact Factor
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    ABSTRACT: Cytoreductive therapy in systemic mastocytosis (SM) includes several drugs whose individual merit has not been well characterized. We retrospectively studied 108 Mayo Clinic patients who met the 2008 WHO diagnostic criteria for SM and received at least one cytoreductive drug. The numbers of patients who were evaluable for response to treatment with interferon-alpha with or without prednisone (IFN-alpha), hydroxyurea (HU), imatinib mesylate (IM) or 2-chlorodeoxyadenosine (2-CdA) were 40, 26, 22, and 22, respectively. The corresponding overall (major) response rates, according to recently published consensus criteria, were 53% (18%), 19% (0%), 18% (9%), and 55% (37%). The respective overall response rates in indolent SM, aggressive SM and SM associated with another clonal hematological nonmast cell lineage disease (SM-AHNMD) were 60%, 60%, 45% for IFN-alpha, 0, 0, 21% for HU, 14%, 50%, 9% for IM and 56%, 50%, 55% for 2-CdA. The absence of mast cell mediator release symptoms in IFN-alpha-treated patients and presence of circulating immature myeloid cells in 2-CdA-treated patients predicted inferior response. TET2 mutational status did not influence treatment response. Although the major response rates with these four cytoreductive agents were still suboptimal and HU was mainly used in patients with SM-AHNMD, the current study favors 2-CdA or IFN-alpha as first-line current therapy in SM and identifies patients who are likely to respond to such therapy.
    American Journal of Hematology 12/2009; 84(12):790-4. DOI:10.1002/ajh.21561 · 3.80 Impact Factor
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    Article: Mast Cells
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    ABSTRACT: Mast cells have been considered for many years to participate specifically in allergic reactions through the release of cytokines, chemokines, proteases, leukotrienes, and bioactive polyamines. Emerging roles for mast cells have been identified recently, which highlight their relevance in both innate and adaptive immunity. Mast cells play a role in many different processes, including clearance of enteric pathogens, food allergies, visceral hypersensitivity, and intestinal cancer. The activation of mast cells can initiate inflammatory reactions that are life-saving in some circumstances (eg, nematode infection) but life-threatening in others (eg, allergy). In recent years, mast cells, their products, and the mechanisms by which mast cell activity can be regulated by the microenvironment are a major area of investigation. The purpose of this review article is to summarize and highlight the latest findings in mast cell biology associated with intestinal homeostasis and pathologies.
    Current Gastroenterology Reports 10/2010; 12(5):349-57. DOI:10.1007/s11894-010-0132-1
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