Tang Ziyu, Hu Hong, Liao Yunguo, Li Chao, Deng Xing, Qiu Shixiang
Department of Interventional Radiology, Nanchong Central Hospital, Sichuan Province, China.
Radiol Case Rep. 2021 Aug 29;16(11):3354-3358. doi: 10.1016/j.radcr.2021.08.004. eCollection 2021 Nov.
Hepatic angiosarcoma is a rare disease, and hepatic hemangiosarcoma with Kasabach-Merritt syndrome (KMS) is even rarer. Although there have been several reports about KMS caused by hepatic angiosarcoma, there has been no mention of successful treatment regimens for hepatic angiosarcoma with KMS. A 64-year-old female patient presented with right upper abdominal pain and multiple cutaneous purpuras for 10 days. Blood analysis revealed that hemoglobin, platelet and fibrinogen were significantly decreased, prothrombin time was prolonged, fibrinogen degradation products were increased. Contrast-enhanced computed tomography scan of the abdomen demonstrated a large mass in the right lobe of the liver, which is pathologically suggestive of hepatic angiosarcoma. Based on the above examination, the patient was diagnosed with KMS caused by hepatic angiosarcoma. Repeated transfusion of blood products could only temporarily improve the coagulation function of the patient. After transarterial chemoembolization, the patient experienced a long-term improvement of blood clotting, and the patient's survival increased by six months. Transarterial chemoembolization should be considered one of effective therapies for hepatic angiosarcoma with KMS.
肝血管肉瘤是一种罕见疾病,而伴有卡萨巴赫-梅里特综合征(KMS)的肝血管内皮肉瘤更为罕见。尽管已有多篇关于肝血管肉瘤导致KMS的报道,但尚未提及针对伴有KMS的肝血管肉瘤的成功治疗方案。一名64岁女性患者出现右上腹疼痛和多处皮肤紫癜10天。血液分析显示血红蛋白、血小板和纤维蛋白原显著降低,凝血酶原时间延长,纤维蛋白原降解产物增加。腹部增强计算机断层扫描显示肝脏右叶有一个大肿块,病理检查提示为肝血管肉瘤。基于上述检查,该患者被诊断为肝血管肉瘤所致KMS。反复输注血液制品只能暂时改善患者的凝血功能。经动脉化疗栓塞后,患者的凝血功能得到长期改善,生存期延长了6个月。经动脉化疗栓塞应被视为治疗伴有KMS的肝血管肉瘤的有效疗法之一。