Selective splenic artery embolization for the treatment of thrombocytopenia and hypersplenism in paroxysmal nocturnal hemoglobinuria.
Selective splenic artery embolization for the treatment of thrombocytopenia and hypersplenism in paroxysmal nocturnal hemoglobinuria.
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DOI：
10.1186/1756-8722-7-27
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发表时间：
2014-03-27
影响因子：
28.5
通讯作者：
Luzzatto L
中科院分区：
文献类型：
--
作者：
Araten DJ;Iori AP;Brown K;Torelli GF;Barberi W;Natalino F;De Propris MS;Girmenia C;Salvatori FM;Zelig O;Foà R;Luzzatto L
PNH与腹部静脉血栓形成有关，可引起脾肿大和脾功能亢进。血栓形成、脾肿大和血小板减少症（TST）的组合具有挑战性，因为需要使用抗凝剂，但血小板减少症可能增加出血风险。脾切除术可减轻血小板减少症，降低门静脉压力，但可引起术后血栓形成和机会性感染。因此，我们试图确定选择性脾动脉栓塞术（SSAE）是否是PNH患者TST脾切除术的安全有效的替代方法。4例PNH和TST患者接受了连续多轮SSAE。通过靶向远端血管进行闭塞，我们的目标是在每次手术中梗死约1/3的脾脏。3/4例患者的血小板计数改善，3/3例患者的腹痛/不适显著改善。一名血小板计数没有反应的患者出现了骨髓衰竭，她在allo-SCT中表现良好。术后疼痛和发热是常见且可管理的;只有一名患者出现了需要引流的分叶状胸腔积液。1例对依库珠单抗仅部分应答的患者对SSAE的应答不仅包括血小板计数的改善，还包括血红蛋白水平的增加和输血需求的减少。这些数据表明，SSAE可以减小脾脏大小和逆转脾功能亢进，而不会使患者暴露于脾切除术的并发症。此外，SSAE可能减少了对依库珠单抗反应有限的患者中调理红细胞的摄取，从而改善了选定患者的生活质量。
PNH is associated with abdominal vein thrombosis, which can cause splenomegaly and hypersplenism. The combination of thrombosis, splenomegaly, and thrombocytopenia (TST) is challenging because anticoagulants are indicated but thrombocytopenia may increase the bleeding risk. Splenectomy could alleviate thrombocytopenia and reduce portal pressure, but it can cause post-operative thromboses and opportunistic infections. We therefore sought to determine whether selective splenic artery embolization (SSAE) is a safe and effective alternative to splenectomy for TST in patients with PNH. Four patients with PNH and TST received successive rounds of SSAE. By targeting distal vessels for occlusion, we aimed to infarct approximately 1/3 of the spleen with each procedure. Three of 4 patients had an improvement in their platelet count, and 3 of 3 had major improvement in abdominal pain/discomfort. The one patient whose platelet count did not respond had developed marrow failure, and she did well with an allo-SCT. Post-procedure pain and fever were common and manageable; only one patient developed a loculated pleural effusion requiring drainage. One patient, who had had only a partial response to eculizumab, responded to SSAE not only with an improved platelet count, but also with an increase in hemoglobin level and decreased transfusion requirement. These data indicate that SSAE can decrease spleen size and reverse hypersplenism, without exposing the patient to the complications of splenectomy. In addition, SSAE probably reduces the uptake of opsonised red cells in patients who have had a limited response to eculizumab, resulting in an improved quality of life for selected patients.
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影响因子：
20.3
作者：
Hillmen, Peter;Muus, Petra;Young, Neal S.
通讯作者：
Young, Neal S.
影响因子：
19.7
作者：
Harned, RK;Thompson, HR;Sokol, RJ
通讯作者：
Sokol, RJ
影响因子：
15.9
作者：
DIXON, RH;ROSSE, WF
通讯作者：
ROSSE, WF
影响因子：
5
作者：
WALLACE, S;GIANTURCO, C;BREE, RL
通讯作者：
BREE, RL
影响因子：
9.6
作者：
Kinjo, N.;Kawanaka, H.;Maehara, Y.
通讯作者：
Maehara, Y.