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

Published

Journal Article

PURPOSE: Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. 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 vincristine in the treatment of KMP. METHODS: We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe. RESULTS: All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability. CONCLUSION: Vincristine presents a safe and sometimes effective treatment option in the management of KMP.

Full Text

Duke Authors

Cited Authors

  • Haisley-Royster, C; Enjolras, O; Frieden, IJ; Garzon, M; Lee, M; Oranje, A; de Laat, PCJ; Madern, GC; Gonzalez, F; Frangoul, H; Le Moine, P; Prose, NS; Adams, DM

Published Date

  • August 2002

Published In

Volume / Issue

  • 24 / 6

Start / End Page

  • 459 - 462

PubMed ID

  • 12218593

Pubmed Central ID

  • 12218593

Electronic International Standard Serial Number (EISSN)

  • 1536-3678

International Standard Serial Number (ISSN)

  • 1077-4114

Digital Object Identifier (DOI)

  • 10.1097/00043426-200208000-00010

Language

  • eng