نتایج جستجو برای: systemic mastocytosis
تعداد نتایج: 175955 فیلتر نتایج به سال:
Mastocytosis is a rare group of disorders with chronic and episodic mast cell release of mediators which can have systemic and cutaneous manifestations. Triggers of anaphylaxis include commonly used medications for anesthesia, analgesia, and muscle relaxation. There is little in the literature regarding local anesthesia in emergent surgery for patients with mastocytosis. This case details the u...
Mastocytosis is a type of myeloproliferative neoplasm characterized by accumulation and proliferation of morphologically and immunophenotypically abnormal mast cells in 1 or more organ systems. Clinical manifestations vary depending upon the organ involved and chemical mediators released by mast cells along with constitutional symptoms and musculoskeletal complaints. We report a case of isolate...
Systemic mastocytosis is a neoplastic proliferation of mast cells that frequently presents with associated clonal hematological non-mast cell lineage disease. Myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene are a heterogenous group of rare and aggressive hematopoietic stem cell disorders. About a dozen of chromosome changes involving the FGFR1 gene, presenting as myeloid or ...
Various translocations involving the PDGFRB gene are identified in myeloid neoplasms. However, the PRKG2/PDGFRB fusion gene associated with t(4;5)(q21;q33) has previously been reported in only 3 patients. We present the case of a 26-year-old woman with microcytic anemia, basophilia, thrombocytosis, and massive splenomegaly, who was found to have systemic mastocytosis and associated clonal hemat...
Systemic mastocytosis (SM) is a rare clonal disorder of mast cells that can range from chronic smouldering type to aggressive mast cell leukaemia. It presents with non-specific symptoms like urticaria pigmentosa, unexplained flushing, hypotension and abdominal cramps, which may lead to a misdiagnosis, if there is no high index of clinical suspicion. This is a case report of a 52-year-old lady, ...
BACKGROUND Indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with reduced quality of life. Masitinib inhibits KIT and LYN kinases that are involved in indolent systemic mastocytosis pathogenesis. We aimed to assess safety and efficacy of masitinib versus placebo in severely symptomatic patients who were unresponsive...
Osteoporotic vertebral fractures are uncommon in young adults and usually indicate an underlying disease. Systemic mastocytosis is a myeloproliferative neoplasm, which can be associated with osteoporosis. A previously healthy 30-year-old man presented with an L4 burst fracture after lifting a heavy object. He was operated with laminectomy and posterior lumbar instrumentation. During surgery, ab...
The prognostic heterogeneity of the World Health Organization category of "systemic mastocytosis with associated clonal hematologic nonmast cell lineage disease" (SM-AHNMD) has not been systematically validated by primary data. Among 138 consecutive cases with SM-AHNMD, 123 (89%) had associated myeloid neoplasm: 55 (45%) myeloproliferative neoplasm (SM-MPN), 36 (29%) chronic myelomonocytic leuk...
BACKGROUND The association of systemic mastocytosis (SM) with a non-mast cell haematological neoplasm represents a specific subtype of mastocytosis termed systemic mastocytosis with associated haematological non-mast cell disease (SM-AHNMD). The overwhelming majority of the associated neoplasms are of myeloid origin, while lymphoid neoplasms associated with SM have been reported rarely. Associa...
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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