Improving cytopenia with splenic artery embolization in a patient with paroxysmal nocturnal hemoglobinuria on eculizumab
Improving cytopenia with splenic artery embolization in a patient with paroxysmal nocturnal hemoglobinuria on eculizumab
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DOI：
10.1007/s12185-013-1454-1
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发表时间：
2013-12-01
影响因子：
2.1
通讯作者：
Kelly, Richard J.
中科院分区：
文献类型：
--
作者：
Krishnan, Suresh K.;Hill, Anita;Kelly, Richard J.
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。我们描述了一个病人治疗下与抗补体抗体依库珠单抗谁开发全血细胞减少症，需要输血，由于巨大的脾肿大。患者接受了两次单独的脾栓塞术，这减少了脾脏的大小，并改善了他的血细胞计数，使其不再需要输血。由于脾切除术后潜在的并发症，特别是血栓形成，选择了脾栓塞术。
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.