title: Clinical analysis of Kasabach-Merritt syndrome in 17 neonates.
abstract: BACKGROUND
  Kasabach-Merritt syndrome (KMS) is characterized by giant hemangiomas and severe thrombocytopenia, which may result in life-threatening multi-organ hemorrhage. This study evaluated the clinical characteristics, treatments, and outcomes in neonates with KMS, in order to find out the optimal therapy.
  
  
  METHODS
  The clinical data of 17 patients treated for KMS in the Department of Neonates, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, China from January 2007 to January 2012 were retrospectively analyzed.
  
  
  RESULTS
  The patients were 13 males and 4 females, aged 17 hours to 28 days at admission. Four patients had visceral hemangiomas and 13 had cutaneous hemangiomas. All had thrombocytopenia and coagulation disorders. Intravenous steroid therapy was initially effective in 6 patients (of which 3 relapsed) and ineffective in 11. The 11 patients with a poor response to steroids and the 3 who relapsed underwent arterial embolization therapy, which was effective in 9 patients (of which 1 relapsed), ineffective in 4, and discontinued before completion in 1. Subsequently, four patients in whom arterial embolization therapy was ineffective and one with relapse were treated with vincristine. This was effective in four patients, and the other died of disseminated intravascular coagulation. Steroid therapy was effective in 35.3% of patients, but the relapse rate was 50%. Arterial embolization was effective in 64.3% of patients and vincristine was effective in 80%.
  
  
  CONCLUSIONS
  In patients with neonatal KMS, steroid therapy has a low rate of effectiveness and high rate of relapse. Arterial embolization has a good rate of effectiveness. Combined steroid and embolization therapy should be considered for first-line treatment of neonatal KMS. If this approach is ineffective, vincristine may be useful.
authors.forename: Ping
authors.initials: P
authors.lastname: Wang
authors.affiliation: Department of Neonatology, Guangzhou Women and Children's Medical Center, Jinan University, Renminzhong Road 318, Guangzhou 510120, China. zhouwei_pu002@126.com.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhou
authors.forename: Li
authors.initials: L
authors.lastname: Tao
authors.forename: Ning
authors.initials: N
authors.lastname: Zhao
authors.forename: Xiao-Wen
authors.initials: XW
authors.lastname: Chen
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D004621:Embolization, Therapeutic / Q000706:statistics & numerical data; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D014750:Vincristine; D003907:Dexamethasone