title: [Is alfa-interferon still current in the management of Kasabach-Merritt syndrome?].
abstract: Kasabach-Merritt syndrome (KMS), characterized by thrombocytopenia, may complicate vascular tumors such as kaposiform hemangioendothelioma and tufted angioma. We report on two infants, respectively 2 months and 15 days old at the onset of symptoms, the first of whom presented with a left cervico-occipito-scapular hemangioma with parotid extension, and the second with a vascular tumor located on the left shoulder with fast extension on the left inferior hemithorax and the left arm. Thrombocytopenia (< 20 G/L) was associated in both cases. Treatment comprised first high-dose corticosteroids (2mg/kg) in association with vincristine (1mg/m(2)/week). Interferon was introduced as third-line treatment at 3 MIU/m(2)/day. In the first patient, interferon was effective both on thrombocytopenia and tumor in 2 months. In the second patient, the first interferon treatment was not effective despite 6 months of therapy. However, a second treatment 8 years later was successful. After 10 and 3 years follow-up, respectively, there were no side effects and most particularly no neurologic complications. These two observations open the discussion of the role of interferon (3 MIU/m(2)/day) as well as new therapies in the control of angiogenesis in case of failure of first-line treatment of complicated KMS vascular tumors.
authors: Inserm, U1111, service de gastroentérologie, hépatologie et nutrition pédiatrique, hospices civils de Lyon, hôpital Femme-Mère-Enfant, CHU de Lyon, université Lyon 1, 59, boulevard Pinel, 69677 Bron, France. Electronic address: remi.duclaux-loras@chu-lyon.fr.
authors: R
authors: R
authors: Duclaux-Loras
authors: Inserm, U1111, service de gastroentérologie, hépatologie et nutrition pédiatrique, hospices civils de Lyon, hôpital Femme-Mère-Enfant, CHU de Lyon, université Lyon 1, 59, boulevard Pinel, 69677 Bron, France.
authors: A
authors: A
authors: Lachaux
authors: Service d'imagerie pédiatrique et fœtale, centre pluridisciplinaire de diagnostic prénatal, hôpital Femme-Mère-Enfant, CHU de Lyon, université Claude-Bernard Lyon I, Bron, France.
authors: L
authors: L
authors: Guibaud
authors: Unité protégée, institut d'hématologie et d'oncologie pédiatrique, centre Léon-Berard, Lyon, France.
authors: Y
authors: Y
authors: Bertrand
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016898:Interferon-alpha / Q000009:adverse effects / Q000627:therapeutic use*; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging; D012008:Recurrence; D019233:Retreatment; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D016898:Interferon-alpha; D014750:Vincristine