Kaposiform Hemangioendothelioma with Kasabach-Merritt phenomenon: Successful Treatment with Vincristine and Ticlopidine

Autor: José Bernabeu-Wittel, Juan Carlos López-Gutiérrez, Inmaculada Bueno-Rodriguez, Román Barabash-Neila, Esther García-Rodríguez, Gemma Ramírez-Villar
Rok vydání: 2011
Předmět:
Zdroj: The Indian Journal of Pediatrics. 79:1386-1387
ISSN: 0973-7693
0019-5456
DOI: 10.1007/s12098-011-0625-4
Popis: Sir, Kaposiform hemangioendothelioma is a rare vascular tumor that often develops Kasabach-Merritt phenomenon. The treatment of this situation is a challenge that is not succesfully solved many times. A 24-d-old-boy was referred from another hospital, for evaluation of a congenital facial tumor. He was born after an uneventful pregnancy via normal spontaneous vaginal delivery. On physical examination, a firm, poorly demarcated, violaceous, warm tumor involved the left cheek area was found (Fig. 1). The lesion progressively enlarged during 4 wk after birth causing upper airway obstruction. Endotracheal intubation followed by tracheostomy was needed. His hemoglobin level at presentation was 9.0 g/dL, and platelets were 38×10/μL. Platelet count decreased down to 16×10/μL, developing a thrombocytopenic consumption coagulopathy, with hypofibrinogenemia (fibrinogen level, 61 mg/dL (reference range, 170–420 mg/dL), that required treatment with endovenous fibrinogen (30mg/kg/daily). Levels of D-dimers >2000 ng/mL (reference range
Databáze: OpenAIRE
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