title: Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach-Merritt phenomenon using four-drug regimen.
abstract: We report a case of a 6-month-old girl suffering from a kaposiform hemangioendothelioma of the chest wall, associated with Kasabach-Merritt phenomenon. Despite rapid intervention with cortisone and interferon alpha the tumor led to a life-threatening clinical condition with progressive growth and consumption coagulopathy under therapy. Because therapy for kaposiform hemangioendotheliomas with a single anti-angiogenic or anti-proliferative agent has not been reported to be very successful, we administered vincristine, combined with cyclophosphamide, actinomycin D, and methotrexate in a critically ill patient. After six cycles of the applied four-drug regiment, the infant was in remission, which has been maintained for 5 months since stopping therapy.
authors.affiliation: Pediatric Haematoloy/Oncology, Dr. v. Haunersches Kinderspital, LMU-University Munich, Munich, Germany. Julia.Hauer@med.uni-muenchen.de
authors.forename: Julia
authors.initials: J
authors.lastname: Hauer
authors.forename: Ulrike
authors.initials: U
authors.lastname: Graubner
authors.forename: Nikos
authors.initials: N
authors.lastname: Konstantopoulos
authors.forename: Susanne
authors.initials: S
authors.lastname: Schmidt
authors.forename: Thomas
authors.initials: T
authors.lastname: Pfluger
authors.forename: Irene
authors.initials: I
authors.lastname: Schmid
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D003348:Cortisone / Q000008:administration & dosage; D016638:Critical Illness; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D005260:Female; D006390:Hemangioendothelioma / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D016898:Interferon-alpha / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D012074:Remission Induction; D013899:Thoracic Neoplasms / Q000188:drug therapy* / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D016898:Interferon-alpha; D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide; D003348:Cortisone; D008727:Methotrexate