Symptomatic response to imatinib mesylate in cutaneous mastocytosis associated with chronic myelomonocytic leukemia.

摘要: Mastocytosis is an uncommon disorder defined by increased and abnormal mast cells in one or more tissues. Cutaneous mastocytosis (cm) limited to the skin, with varying degrees of rash, pruritus, disfigurement. Systemic (sm) typically involves bone marrow, sometimes association other marrow disorders, including chronic myelomonocytic leukemia (cmml). has been associated somatic mutations gene encoding tyrosine kinase Kit, leading identification Kit as a therapeutic target. The inhibitor imatinib mesylate approved for aggressive sm. We present unusual patient disabling pruritus from telangiectasia macularis eruptiva perstans, subtype cm, cmml, but no evidence systemic cell disease. She was treated experienced marked improvement her pruritus. Concomitant cm cmml have not previously reported, report first successful therapy adult cm.