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Kasabach-Merritt syndrome caused by giant hemangiomas of the spleen in patients with Proteus syndrome.

Abstract
Proteus syndrome is a rare hamartomous disorder with highly variable manifestations of disproportionate overgrowth of body parts, epidermal nevi and vascular malformations. We describe two patients who had typical clinical and imaging Proteus syndrome features. One of most characteristic findings in these two patients was that they had giant hemangiomas of the spleen that caused Kasabach-Merritt syndrome, demonstrating clinical bleeding and disseminated intravascular coagulation laboratory alterations. The present two cases are the first report of this complication in Proteus syndrome. The patients' bleeding tendency and hemostatic defects were completely corrected after they successfully underwent splenectomy.
AuthorsZhaoyue Wang, Ziqiang Yu, Yanhua Su, Haiyan Yang, Lijuan Cao, Xiaojuan Zhao, Hao Hu, Shenghua Zhan, Changgeng Ruan
JournalBlood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis (Blood Coagul Fibrinolysis) Vol. 18 Issue 5 Pg. 505-8 (Jul 2007) ISSN: 0957-5235 [Print] England
PMID17581327 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Hemostatics
Topics
  • Adolescent
  • Adult
  • Angiomatosis (pathology, therapy)
  • Disseminated Intravascular Coagulation
  • Female
  • Hemangioma (pathology, therapy)
  • Hemostatics (administration & dosage)
  • Humans
  • Neoplasms, Second Primary (pathology, therapy)
  • Proteus Syndrome (pathology, therapy)
  • Splenectomy
  • Splenic Neoplasms (pathology, therapy)

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