title: Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.
abstract: OBJECTIVE
  Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP.
  
  
  METHODS
  We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe.
  
  
  RESULTS
  All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability.
  
  
  CONCLUSIONS
  Vincristine presents a safe and sometimes effective treatment option in the management of KMP.
authors.affiliation: Duke University Medical Center, Durham, NC, USA.
authors.forename: Camille
authors.initials: C
authors.lastname: Haisley-Royster
authors.forename: Odile
authors.initials: O
authors.lastname: Enjolras
authors.forename: Ilona J
authors.initials: IJ
authors.lastname: Frieden
authors.forename: Maria
authors.initials: M
authors.lastname: Garzon
authors.forename: Margaret
authors.initials: M
authors.lastname: Lee
authors.forename: Arnold
authors.initials: A
authors.lastname: Oranje
authors.forename: Peter C J
authors.initials: PC
authors.lastname: de Laat
authors.forename: Gerard C
authors.initials: GC
authors.lastname: Madern
authors.forename: Francisco
authors.initials: F
authors.lastname: Gonzalez
authors.forename: Hayder
authors.initials: H
authors.lastname: Frangoul
authors.forename: Philippe
authors.initials: P
authors.lastname: Le Moine
authors.forename: Neil S
authors.initials: NS
authors.lastname: Prose
authors.forename: Denise M
authors.initials: DM
authors.lastname: Adams
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D005260:Female; D005340:Fibrinogen / Q000378:metabolism; D006390:Hemangioendothelioma / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D018324:Hemangioma, Capillary / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010976:Platelet Count; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D014750:Vincristine / Q000627:therapeutic use*
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D014750:Vincristine; D005340:Fibrinogen