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Kasabach-Merritt Phenomenon: A Retrospective Study of Treatment with Vincristine

2002; Lippincott Williams & Wilkins; Volume: 24; Issue: 6 Linguagem: Inglês

10.1097/00043426-200208000-00010

1536-3678

Camille Haisley-Royster, O Enjolras, Ilona J. Frieden, Maria C. Garzón, Margaret Lee, Peter C. J. de Laat, Gerard C. Madern, Francisco Javier Díaz González, Hayder Frangoul, Philippe Le Moine, Neil S. Prose, Denise M. Adams,

Vascular Malformations Diagnosis and Treatment

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.