[Proteus syndrome with a giant hemangiomas in the spleen associated with chronic DIC--two case report and literature review].
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.
Wang
Zhao-Yue
ZY
Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215006, China.
Su
Yan-Hua
YH
Yang
Hai-Yan
HY
Yu
Zi-Qiang
ZQ
Cao
Li-Juan
LJ
Zhao
Xiao-Juan
XJ
Hu
Hao
H
Zhan
Sheng-Hua
SH
Ruan
Chang-Geng
CG
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