Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with kasabach-merritt phenomenon
- 22 April 2013
- journal article
- clinical trial
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 60 (9), 1478-1481
- https://doi.org/10.1002/pbc.24543
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.This publication has 25 references indexed in Scilit:
- Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 ReferralsThe Journal of Pediatrics, 2013
- Kasabach-Merritt syndrome with large cutaneous vascular tumorsJournal of Indian Association of Pediatric Surgeons, 2012
- Expression of Prox1, Lymphatic Endothelial Nuclear Transcription Factor, in Kaposiform Hemangioendothelioma and Tufted AngiomaThe American Journal of Surgical Pathology, 2010
- Successful Treatment of Kaposiform Hemangioendothelioma and Tufted Angioma With VincristineJournal of Pediatric Hematology/Oncology, 2010
- Management of Stroke in Infants and ChildrenStroke, 2008
- Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenonJournal of the American Academy of Dermatology, 2005
- Residual lesions after Kasabach-Merritt phenomenon in 41 patientsJournal of the American Academy of Dermatology, 2000
- Infants with Kasabach-Merritt syndrome do not have “true” hemangiomasThe Journal of Pediatrics, 1997
- A Randomized Trial Comparing Ticlopidine Hydrochloride with Aspirin for the Prevention of Stroke in High-Risk PatientsThe New England Journal of Medicine, 1989
- Use of aspirin and dipyridamole in children with platelet trapping syndromesThe Journal of Pediatrics, 1983