Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon
1997; Alan R. Liss, Inc.; Volume: 28; Issue: 5 Linguagem: Inglês
10.1002/(sici)1096-911x(199705)28
ISSN1096-911X
AutoresGiovanni Deb, Alessandro Jenkner, Luigi De Sio, Renata Boldrini, C. Bosman, Nicola Standoli, Alberto Donfrancesco,
Tópico(s)Tuberous Sclerosis Complex Research
ResumoA two-month-old infant developed a vascular tumor of the right flank which upon biopsy proved to be a spindle cell hemangioendothelioma. The increased capillary bed characterizing the neoplasm caused a severe thrombocytopenia together with a consumption coagulopathy (Kasabach-Merritt syndrome). The patient, who was dependent on platelet transfusions, improved quickly after interferon alpha-2a was given at the dosage of 3,000,000 U/m2, with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment.
Referência(s)