What Is Malignant Systemic Mastocytosis?


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Malignant systemic mastocytosis is a rare form of mastocytosis that affects the entire body, according to Cancer.net. It is a subgroup of mast cell neoplasms and is often very difficult to diagnose, according to PubMed.

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When malignant systemic mastocytosis occurs, mast cells accumulate in one or many organs throughout the body, according to Cancer.net. Some of the organs commonly affected by this rare disorder include the gastrointestinal tract, liver, spleen, bone marrow and lymph nodes. In many cases, urticaria pigmentosa develops before malignant systemic mastocytosis occurs. This disorder has a 7 percent risk of becoming cancerous when contracted in childhood and an increased risk of up to 30 percent in adults.

General symptoms common to individuals who suffer from mastocytosis include hives, rashes, diarrhea, reddening of the face and shortness of breath, according to Cancer.net. Psychological changes such as difficulty concentrating or increased irritability can also occur. Other symptoms unique to this particular type of mastocytosis include skin lesions, nausea, headache, heart palpitations, anemia and bone pain. Typically, these symptoms manifest in sudden onsets usually described as attacks, with multiple symptoms manifesting at the same time, leaving the patient feeling lethargic and tired afterwards. Treatments include targeted therapy, stem cell transplantation and chemotherapy.

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