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Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon

✍ Scribed by Deb, Giovanni; Jenkner, Alessandro; De Sio, Luigi; Boldrini, Renata; Bosman, Cesare; Standoli, Nicola; Donfrancesco, Alberto


Publisher
John Wiley and Sons
Year
1997
Tongue
English
Weight
424 KB
Volume
28
Category
Article
ISSN
0098-1532

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✦ Synopsis


A 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 ␣-2a was given at the dosage of 3.000.000 U/m 2 , with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment. Med.


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