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Keywords:

  • Kasabach–Merritt phenomenon;
  • kaposiform hemangioendothelioma;
  • tufted angioma;
  • antiaggregant therapy

Abstract

Background

. This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach–Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).

Procedure

. A retrospective review was conducted of medical records of all children with diagnosis of KHE or TA associated with KMP treated with vincristine–aspirin–ticlopidine (VAT) therapy at two different institutions in the same country from 1994 to 2011. Clinical features, response to VAT therapy and outcomes were recorded.

Results

. Eleven patients (mean age 11 months, range 0–36), including seven females (64%) and four males (36%), were identified. Seven patients underwent incisional biopsy and two different histologies were found, KHE in four patients and TA in three patients. Tumors were located in the head and neck (n = 5), chest wall (n = 2), arm (n = 2) and retroperitoneum (n = 2). Mean platelet level was 10,200/mm3 (range 4,000–21,000). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation (63%). All patients underwent VAT therapy. Mean duration of treatment was 3.9 months for vincristine, 13.9 months for aspirin, and 13.4 months for ticlopidine. All patients are alive with a mean follow-up of 4.5 years (range, 2–17).

Conclusions

. Antiaggregant therapy is helpful in combination with vincristine in the treatment of KMP associated with KHE and TA. Prognosis is excellent if severe thrombocytopenia is controlled despite failure in reduction of tumor size. Pediatr Blood Cancer 2013;60:1478–1481. © 2013 Wiley Periodicals, Inc.