Retrospective analysis of Kasabach-Merritt phenomenon: a report of 39 cases

Abstract

Objective To explore the clinical features of Kasabach-Merritt phenomenon (KMP) to provide rationales clinical diagnosis and treatment. Methods From January 2014 to October 2017, the clinical data were analyzed retrospectively for 39 cases of KMP. Results All cases were accordant to the diagnostic criteria of KMP. The therapies included hormone (n=6, 15.4%), intravascular embolism plus hormone (n=18, 46.1%), hormone plus local hardening (n=7), hormone plus vincristine chemotherapy (n=2) and sirolimus (n=6). And the number of outcomes was zero, 4, 1 and 1. The number of lost follow-up was zero, 1, 1 and 1 respectively. Among six cases of sirolimus users, 5 co-users of propranolol and one case of intravascular embolism responded poorly. Five had no recurrence and 1 lost follow-up. KMP was pathologically confirmed in 19 cases. During a follow-up period of 3-45 months, the number of tumor survivors accounted for 38.5%(15/39). Conclusions The method of continuous multimode therapy is preferred for KMP children. Hormone therapy is offered based upon the size and location of tumor. Other options include local injection plus hormone therapy and intravascular embolism plus hormone therapy. Use of sirolimus is indicated for hormonal refractory KMP. Key words: Hemangioendothelioma; Thrombocytopenia