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

Abstract

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, <215 ng/mL). The diagnosis of Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP) was made basing on clinical and laboratory findings. Biopsy of the tumor could not be performed because of the potential for hemorrhage. We implemented a therapeutic regimen with endovenous prednisone (5 mg/kg daily) and vincristine (0.05 mg/kg/ wkly), oral ticlopidine plus aspirin (10 mg/kg/daily). Within 3 wk of treatment the platelet count normalized to 120×10/ μL and the tumor considerably diminished in size. After 3 mo follow-up, the patient was still doing well, with normal platelet counts, a minimal residual vascular lesion (Fig. 2), and 100% performance status. Vincristine was maintained during 11 mo until the stability of the lesion was achieved. At the present time the patient is not receiving any treatment and there is no recurrence after 18 mo of follow-up. KHE is an infrequent vascular neoplasm that usually appears in infancy or can present at birth. The skin is the most frequently affected location followed by soft tissue, and bone infiltration due to its locally aggressive behavior [1, 2]. Consumption coagulopathy (KMP) is a frequent expression of this neoplasm. Its presence should arise the suspicion of this vascular tumor [3]. The prognosis depends on tumor location, size, presence of deeper tissues R. Barabash-Neila (*) : J. Bernabeu-Wittel Pediatric Dermatology Unit, Virgen del Rocio Universitary Hospital, Avda. Manuel Siurot, s/n, 41013 Sevilla, Spain e-mail: romanbarabash@hotmail.com