[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.

CONCLUSION:

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: Zhao-Yue Wang, Yan-Hua Su, Hai-Yan Yang, Zi-Qiang Yu, Li-Juan Cao, Xiao-Juan Zhao, Hao Hu, Sheng-Hua Zhan, Chang-Geng Ruan
• Journal: Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi (Zhonghua Xue Ye Xue Za Zhi) Vol. 28 Issue 3 Pg. 152-5 (Mar 2007) ISSN: 0253-2727 [Print] China
• PMID: 17649705 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Disseminated Intravascular Coagulation (etiology)
• Female
• Hemangioma, Cavernous (complications, diagnostic imaging, surgery)
• Humans
• Proteus Syndrome (complications)
• Splenectomy
• Splenic Neoplasms (complications, diagnostic imaging, surgery)
• Ultrasonography