Kasabach-merritt phenomenon: a retrospective study of treatment with XXXX .

@entity1 @entity2002 @entity455 @entity24428 @entity427 @entity882 @entity2695 @entity75 @entity5 @entity146 @entity1003 @entity1891 @entity490 @entity24429 @entity8146 @entity2106
PURPOSE: Kasabach-Merritt phenomenon (KMP) is characterized by profound @entity882 , @entity2106 , a @entity1003 , and an enlarging @entity455 . The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of @entity1891 in the treatment of KMP. METHODS: We retrospectively reviewed the clinical and laboratory data of 15 @entity1 with KMP treated with @entity1891 at 9 institutions across the United States, South America, and Europe. RESULTS: All 15 @entity1 had profound @entity882 and consumption of @entity2002 at presentation. Ten @entity1 had biopsies of their lesions, and results included five (33.3%) kaposiform @entity8146 , three (20%) tufted @entity2695 , @entity75 (6.7%) with features of both @entity24428 and @entity24429 , and one (6.7%) unclassified @entity5 . All 15 @entity1 had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of @entity1891 therapy. Thirteen @entity1 had an increase in @entity2002 level of 50 mg/dL with an average response time of 3.4 weeks. In 13 @entity1 there was a significant decrease in the size of the @entity455 . The average duration of treatment was 21.5 (+/-12.6) weeks. Four @entity1 (26%) relapsed. All four were successfully treated with a second course of @entity1891 . Complications included one @entity1 with @entity490 , one @entity1 with transient @entity427 , and one @entity1 with @entity146 . CONCLUSION: @entity1891 presents a safe and sometimes effective treatment option in the management of KMP.
