FIP1L1/PDGFRα-Associated Systemic Mastocytosis
نویسندگان
چکیده
منابع مشابه
FIP1L1/PDGFR -Associated Systemic Mastocytosis
Since the identification of the FIP1L1/PDGFRA fusion gene as a pathogenic cause of the hypereosinophilic syndrome (HES), the importance of the molecular classification of HES leading to the diagnosis of chronic eosinophilic leukemia (CEL) has been recognized. As a result, a new category, ‘myeloid and lymphoid neoplasm with eosinophilia and abnormalities in PDGFRA, PDGFRB or FGFR1’, has recently...
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Mastocytosis comprises a spectrum of disorders characterized by abnormal growth of mast cells (MS). Four entities are recognizable according to WHO classification. Association of systemic mastocytosis (SM) with a chronic myeloproliferative neoplasia (SM-AHNMD) is the second frequently category. Published descriptions of the clinicopathologic features of SM-AHNMD are largely limited to individua...
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Systemic mastocytosis (SM) is a condition associated with a clonal neoplastic proliferation of mast cells. Approximately 40% of patients with SM present with an associated clonal hematological non-mast cell lineage disorder. Patients presenting with SM-acute myeloid leukemia (AML) have the worst prognosis. We present a case of a 62-year-old woman who was diagnosed with SM-AML. After initial tre...
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circular macules, less than 1 cm in diameter, located on his trunk and extremities [Figure A]. Wheals and surrounding erythema developed in the lesions after rubbing (positive Darier sign). Laboratory analyses showed mild eosinophilia with an increased total tryptase level of 40 ng/ml. Densitometry was normal. With the strong clinical suspicion of cutaneous mastocytosis, a skin biopsy was perfo...
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Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast cells in one or more organ systems. SM in association with plasma cell dyscrasia is very rare. We report a first case of indolent SM (ISM) associated with light chain deposition disease (LCDD) in a kidney biopsy from a 59-year-old female presenting with skin rash, elevated serum creatinine, hematuria and mild protein...
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ژورنال
عنوان ژورنال: International Archives of Allergy and Immunology
سال: 2010
ISSN: 1423-0097,1018-2438
DOI: 10.1159/000312134