Department of Hematology/Oncology, St-James University Hospital, Beckett Street, Leeds, LS9 7TF, UK,
Int J Hematol. 2013 Dec;98(6):716-8. doi: 10.1007/s12185-013-1454-1. Epub 2013 Oct 25.
Paroxysmal nocturnal hemoglobinuria is a rare acquired stem cell disorder characterized by intravascular hemolysis, aplasia and an increased risk of thrombosis. We describe a patient under treatment with the anti-complement antibody eculizumab who developed pancytopenia, requiring blood transfusions, due to massive splenomegaly. The patient underwent two separate splenic embolizations, which reduced the size of the spleen and improved his blood count to the point that blood transfusions were no longer necessary. Splenic embolization was chosen over splenectomy due to the potential postoperative complications of splenectomy, especially that of thrombosis.
阵发性睡眠性血红蛋白尿症是一种罕见的获得性干细胞疾病,其特征是血管内溶血、再生障碍和血栓形成风险增加。我们描述了一位接受抗补体抗体依库珠单抗治疗的患者,由于巨脾导致全血细胞减少,需要输血。患者接受了两次单独的脾动脉栓塞术,这降低了脾脏的大小,并使他的血细胞计数得到改善,以至于不再需要输血。由于脾切除术的潜在术后并发症,特别是血栓形成,选择了脾动脉栓塞术而不是脾切除术。