title: 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.
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu Province 215006, China. w950831@public1.sz.js.cn
authors.forename: Zhaoyue
authors.initials: Z
authors.lastname: Wang
authors.forename: Ziqiang
authors.initials: Z
authors.lastname: Yu
authors.forename: Yanhua
authors.initials: Y
authors.lastname: Su
authors.forename: Haiyan
authors.initials: H
authors.lastname: Yang
authors.forename: Lijuan
authors.initials: L
authors.lastname: Cao
authors.forename: Xiaojuan
authors.initials: X
authors.lastname: Zhao
authors.forename: Hao
authors.initials: H
authors.lastname: Hu
authors.forename: Shenghua
authors.initials: S
authors.lastname: Zhan
authors.forename: Changgeng
authors.initials: C
authors.lastname: Ruan
mesh_terms: D000293:Adolescent; D000328:Adult; D000798:Angiomatosis / Q000473:pathology* / Q000628:therapy; D004211:Disseminated Intravascular Coagulation; D005260:Female; D006391:Hemangioma / Q000473:pathology* / Q000628:therapy; D006490:Hemostatics / Q000008:administration & dosage; D006801:Humans; D016609:Neoplasms, Second Primary / Q000473:pathology* / Q000628:therapy; D016715:Proteus Syndrome / Q000473:pathology* / Q000628:therapy; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000473:pathology* / Q000628:therapy
chemical_list: D006490:Hemostatics