Long-lasting complete response to @entity2213 in a XXXX with systemic mastocytosis exhibiting wild type KIT.

@entity1 @entity262 @entity1583 @entity6339 @entity272 @entity5311 @entity2213
@entity262 ( @entity262 ) is a @entity1583 characterized by abnormal expansion of mast cells (MCs) in visceral organs. The skin is involved in most cases. In adult @entity1 the transforming KIT mutation @entity5311 is usually present and confers resistance against @entity2213 . Therefore, @entity2213 is not recommended for @entity1 with KIT @entity5311 + @entity262 . Nonetheless, @entity2213 may work in @entity1 with @entity262 lacking KIT @entity5311 . However, little is known about long-term efficacy and safety of this drug in @entity262 . We report on a 62-year-old female @entity1 with indolent @entity262 (ISM) who suffered from severe debilitating skin involvement despite therapy with anti-mediator-type drugs, @entity6339 and ultraviolet-A-radiation. Although multifocal MC infiltrates were detected in the bone marrow by immunohistochemistry, no KIT mutation was found by sequencing analysis. In 2003, treatment with @entity2213 (induction, 400 mg/day; maintenance, 200 mg/day) was initiated. During therapy, @entity272 and tryptase levels decreased. Treatment was well tolerated without any side effects. After 10 years, @entity272 have disappeared and the tryptase level is within normal range. This case-study confirms the long-term efficacy and safety of @entity2213 in @entity1 with @entity262 lacking activating KIT mutations. @entity2213 should be considered in select cases of @entity262 in whom MCs exhibit wild-type KIT.
