Kasabach-Merritt syndrome caused by giant hemangiomas of the spleen in patients with Proteus syndrome.
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.
Wang
Zhaoyue
Z
Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu Province 215006, China. w950831@public1.sz.js.cn
Yu
Ziqiang
Z
Su
Yanhua
Y
Yang
Haiyan
H
Cao
Lijuan
L
Zhao
Xiaojuan
X
Hu
Hao
H
Zhan
Shenghua
S
Ruan
Changgeng
C
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
D006490:Hemostatics