Culafić Dj, Miljić P, Perisić M, Djuranović S, Popović D, Krstić M, Jovanović I, Pavlović A, Mijalković N, Sokić-Milutinović A, Pesko P
Institut za bolesti digestivnog sistema, KCS, Beograd.
Acta Chir Iugosl. 2007;54(1):83-9. doi: 10.2298/aci0701083c.
Thrombosis of portal and hepatic veins is one of the most severe complications and most important causes of death of patients with chronic myeloproliferative diseases. Based on results of the past studies, myeloproliferative diseases were the causes of hepatic veins thrombosis in 30% and portal vein thrombosis in 20% of patients. The study presented 4 patients with myeloproliferative diseases complicated by thrombosis of splanchnic veins, aiming at the illustration of issue complexity in diagnostics and therapy. Two patients with portal vein thrombosis and recurring hemorrhage from esophageal varicosity were described. The first case was planned for shunting, while another case sustained bleeding on what account his anticoagulant therapy was discontinued, but it caused mesenterial thrombosis resulting in lethal outcome. Another two patients had hepatic veins thrombosis. Due to frequent, life-threatening bleeding from the esophageal and gastric varices, a patient with chronic Budd-Chiari syndrome and lineal vein thrombosis underwent mesocaval shunting. An immediate postoperative period was manifested by multiple thrombosis and hemorrhages that ended in his death. A patient with the acute Budd-Chiari syndrome was administered myelosuppressants and anticoagulants on time so reperfusion was restored. In myeloproliferative diseases, thrombosis of portal and hepatic veins gives rise to excessive portal hypertension with profuse hemorrhage from the esophageal and gastric varicosity which is difficult to manage because of complex coagulation disorders.
门静脉和肝静脉血栓形成是慢性骨髓增殖性疾病患者最严重的并发症之一,也是最重要的死亡原因。根据以往研究结果,骨髓增殖性疾病是30%的肝静脉血栓形成患者和20%的门静脉血栓形成患者的病因。该研究报告了4例骨髓增殖性疾病合并内脏静脉血栓形成的患者,旨在阐明诊断和治疗中的问题复杂性。描述了2例门静脉血栓形成且伴有食管静脉曲张反复出血的患者。第一例计划进行分流手术,而另一例因持续出血而停用抗凝治疗,但这导致肠系膜血栓形成,最终导致致命后果。另外2例患者有肝静脉血栓形成。由于食管和胃静脉曲张频繁发生危及生命的出血,一名患有慢性布加综合征和线性静脉血栓形成的患者接受了肠系膜上腔静脉分流术。术后即刻出现多处血栓形成和出血,最终导致患者死亡。一名急性布加综合征患者及时接受了骨髓抑制药物和抗凝剂治疗,从而恢复了再灌注。在骨髓增殖性疾病中,门静脉和肝静脉血栓形成会导致门静脉高压过度,伴有食管和胃静脉曲张大量出血,由于复杂的凝血障碍,这种出血难以处理。