肝脏Kasabach-Merritt 综合征1例报告及文献复习

Chinese Journal of Practical Surgery ›› 2013, Vol. 33 ›› Issue (05) : 400-403.

Chinese Journal of Practical Surgery ›› 2013, Vol. 33 ›› Issue (05) : 400-403.
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Abstract

Hepatic Kasabach-Merritt syndrome: A case report and literature review        LIN Han, MA Yong, JIANG Hong-chi, et al. Department of Hepatic Surgery, the First Affiliated Hospital of Harbin Medical University; Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin 150001, China
Corresponding author: LIU Lian-xin, E-mail: liulianxin@medmail.com.cn
Abstract    Objective    To investigate the clinical features, diagnosis and treatments of hepatic Kasabach-Merritt Syndrome (KMS).  Methods    One case of hepatic KMS admitted in October 2009 in the First Affiliated Hospital of Harbin Medical University and the literature (50 cases) reported from 1984 to 2010 were collected. The incidence, clinical manifestations, laboratory tests, treatments and prognosis were analyzed retrospectively.  Results    The pathophysiological basis of KMS is thrombocytopenia which is closely related to the giant hemangioma and disseminated intravascular coagulation (DIC). The clinical manifestations of the tumor are diversified and pathological manifestations are mainly cavernous hemangioma and hemangioendothelioma. Treatment procedure includes the correction of DIC and thrombocytopenia, surgical treatment (hepatectomy, liver transplantation, etc.)or pharmacotherapy (glucocorticoids, interferon, etc.).  Conclusion    Hemangioma, especially giant hemangioma with thrombocytopenia, should be alert to hepatic KMS. Once the diagnosis is confirmed, the hepatic KMS should be given combined modality therapy according to the size and location of the tumor, the core of which is surgery and interventional therapy, and assisted by a variety of treatment modalities, such as pharmacotherapy, blood transfusions, etc.

Key words

Kasabach-Merritt syndrome / liver / hemangioma / thrombocytopenia

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