title: [Proteus syndrome with a giant hemangiomas in the spleen associated with chronic DIC--two case report and literature review].
abstract: OBJECTIVE
  To investigate the clinical manifestations, pathologic features and laboratory findings in two Proteus syndrome patients with giant hemangiomas in the spleen and chronic DIC.
  
  
  METHODS
  Ultrasound imaging and magnetic resonance imaging (MRI) were used for analysing the characteristics of the giant hemangiomas in the spleen. The spleen specimen was examined pathologically for the feature of the hemangioma. Homostatic tests were performed by routine laboratory methods.
  
  
  RESULTS
  Two Proteus syndrome patients with giant hemangiomas in the spleen causing chronic DIC (Kasabach-Merritt syndrome) were first reported. They were recovered after splenectomy.
  
  
  CONCLUSIONS
  Proteus syndrome when accompanied giant hemangioma could cause chronic DIC. Significantly decreased plasma fibrinogen level in this case might be helpful for the differential diagnosis from DIC caused by other diseases.
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215006, China.
authors.forename: Zhao-Yue
authors.initials: ZY
authors.lastname: Wang
authors.forename: Yan-Hua
authors.initials: YH
authors.lastname: Su
authors.forename: Hai-Yan
authors.initials: HY
authors.lastname: Yang
authors.forename: Zi-Qiang
authors.initials: ZQ
authors.lastname: Yu
authors.forename: Li-Juan
authors.initials: LJ
authors.lastname: Cao
authors.forename: Xiao-Juan
authors.initials: XJ
authors.lastname: Zhao
authors.forename: Hao
authors.initials: H
authors.lastname: Hu
authors.forename: Sheng-Hua
authors.initials: SH
authors.lastname: Zhan
authors.forename: Chang-Geng
authors.initials: CG
authors.lastname: Ruan
mesh_terms: D000293:Adolescent; D004211:Disseminated Intravascular Coagulation / Q000209:etiology*; D005260:Female; D006392:Hemangioma, Cavernous / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D016715:Proteus Syndrome / Q000150:complications*; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D014463:Ultrasonography