Rosen Emily A, Vallurupalli Mounica, Choy Edwin, Lennerz Jochen K, Kuter David J
Harvard Medical School.
Dana-Farber/Partners Cancer Center.
Medicine (Baltimore). 2018 Nov;97(47):e13321. doi: 10.1097/MD.0000000000013321.
Hepatic angiosarcoma is a rare endothelial cell tumor that may lead to concurrent consumptive coagulopathies including disseminated intravascular coagulation (DIC). This report details a multifaceted approach to managing DIC in a patient with advanced-stage hepatic angiosarcoma, which continued to progress after a brief response to taxane-based chemotherapy.
A 55-year-old man with a recent history of hemorrhoids and hemarthroses presented with acute rectal bleeding. He was found to have concurrent hepatomegaly, abnormal liver function tests, anemia, thrombocytopenia, and coagulopathy.
DIC in the setting of hepatic angiosarcoma.
The patient's acute bleeding in the setting of DIC was controlled with a combination of antifibrinolytic agents to prevent clot breakdown, heparin products to prevent deposition of new clot, and romiplostim to increase platelet production. His angiosarcoma was treated with various combinations of chemotherapy, including taxane-based chemotherapy, doxorubicin, and pazopanib.
The patient's DIC and acute bleeding on initial presentation improved following treatment with unfractionated heparin and low-molecular weight heparin maintenance therapy. It is unclear if the chemotherapy to treat the hepatic angiosarcoma played a significant role in the improvement of DIC.
Laboratory measurement of prothrombin fragment 1.2, a byproduct of prothrombin conversion to thrombin, proved to be a useful way to monitor this patient's DIC over time.
肝血管肉瘤是一种罕见的内皮细胞肿瘤,可能导致并发消耗性凝血病,包括弥散性血管内凝血(DIC)。本报告详细介绍了一名晚期肝血管肉瘤患者并发DIC的多方面管理方法,该患者在对紫杉烷类化疗有短暂反应后病情仍继续进展。
一名55岁男性,近期有痔疮和关节积血病史,出现急性直肠出血。发现他同时伴有肝肿大、肝功能检查异常、贫血、血小板减少和凝血病。
肝血管肉瘤合并DIC。
通过联合使用抗纤溶药物以防止血栓分解、肝素类产品以防止新血栓形成以及罗米司亭以增加血小板生成,控制了该患者在DIC情况下的急性出血。他的血管肉瘤接受了多种化疗方案的治疗,包括紫杉烷类化疗、多柔比星和帕唑帕尼。
使用普通肝素和低分子肝素维持治疗后,患者初诊时的DIC和急性出血情况有所改善。尚不清楚治疗肝血管肉瘤的化疗是否在DIC的改善中发挥了重要作用。
凝血酶原片段1.2(凝血酶原转化为凝血酶的副产物)的实验室检测被证明是长期监测该患者DIC的一种有用方法。