Disease Information for Mastocytosis, systemic: Definition

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  • Mastocytic tissue accumulation with unknown cause; released histamine and various leukotrienes account for the clinical syndrome; usually accompanied by peptic ulcer and diarrhea syndromes and urticaria pigmentosa; attacks are triggered by drugs [aspirin,opiates,codeine] and alcohol as well as stress, exercise, and temperature extremes, also specific foods including lobster and aged cheese;----------------

    Mastocytosis; Systemic Mast Cell Disease; Systemic Mastocytosis; Urticaria pigmentosa; Aggressive Systemic Mastocytosis; Cutaneous Mastocytosis; Indolent Systemic Mastocytosis; Mast Cell Leukemia; Mast Cell Sarcoma/Extracutaneous Mastocytoma; Mastocytosis with an Associated Hematological Disorder; Mastocytosis is a rare disorder characterized by abnormal accumulations of mast cells in skin, bone marrow, and internal organs such as the liver, spleen and lymph nodes; Cases beginning during adulthood tend to involve the inner organs in addition to the skin whereas, during childhood, the condition is often marked by skin manifestations with minimal or no organ involvement; When there is evidence of bone marrow or internal organ involvement, the disease is referred to as "systemic mastocytosis"; Although the majority of cases follow an indolent course, some patients may have evidence of a blood disorder such as a myelodysplastic or myeloproliferative disorder at the time of diagnosis; The course and prognosis of mastocytosis in these patients are determined by this associated hematologic disorder; More aggressive forms of mastocytosis and mast cell leukemias are very rarely encountered---[NORD]-------------

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