title: Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon.
abstract: 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 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.
authors.affiliation: Department of Pediatric Oncology, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Deb
authors.forename: A
authors.initials: A
authors.lastname: Jenkner
authors.forename: L
authors.initials: L
authors.lastname: De Sio
authors.forename: R
authors.initials: R
authors.lastname: Boldrini
authors.forename: C
authors.initials: C
authors.lastname: Bosman
authors.forename: N
authors.initials: N
authors.lastname: Standoli
authors.forename: A
authors.initials: A
authors.lastname: Donfrancesco
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D005260:Female; D006390:Hemangioendothelioma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D011994:Recombinant Proteins; D012878:Skin Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications*
chemical_list: D000970:Antineoplastic Agents; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins