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大龄儿童的肝脏间叶性错构瘤:与弥散性血管内凝血的关联。

Mesenchymal hamartoma of the liver in an older child: association with disseminated intravascular coagulation.

作者信息

Rao S P, Bhagavath S, Chen C K, Tolete-Velcek F

出版信息

Med Pediatr Oncol. 1984;12(2):112-5. doi: 10.1002/mpo.2950120210.

Abstract

A 9-year-old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which re-resulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported before.

摘要

一名9岁黑人女孩因肝脏右叶的巨大原发性肿瘤接受了右肝切除术。组织病理学检查确诊为间叶性错构瘤,这是一种罕见的良性肿瘤,主要发生在2岁以下儿童。术前,患者有轻度弥散性血管内凝血(DIC)的实验室证据,但无出血表现。为手术做准备时,患者接受了全血置换输血和血小板输血,这使止血参数有了显著改善。尽管手术过程中失血似乎得到了充分补充,但患者在手术接近尾声时突然心脏骤停并死亡。可能的死亡原因是低血容量。这例间叶性肝错构瘤病例显示了两个不寻常的特征:患者年龄以及与DIC的关联。据我们所知,后者此前尚未有过报道。

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