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遗传性球形红细胞增多症患者行全脾切除术或部分脾切除术后的临床及实验室检查结果

Clinical and laboratory outcomes following total or partial splenectomy in patients with hereditary spherocytosis.

作者信息

Tripodi Serena I, Shamberger Robert C, Heeney Matthew M, Tubman Venée N

机构信息

Department of Pediatrics, University of Brescia , Brescia , Italy.

Department of Surgery, Boston Children's Hospital , Boston , Massachusetts , USA.

出版信息

Pediatr Hematol Oncol. 2019 Sep;36(6):382-389. doi: 10.1080/08880018.2019.1637983. Epub 2019 Jul 26.

Abstract

This study compared outcomes following total (TS) or partial splenectomy (PS) among patients with hereditary spherocytosis. Seventy-nine patients (TS = 33, PS = 46) were identified. The follow-up period was longer after PS (59.6 vs. 24.9 months,  < .001). Long-term adverse events occurred more frequently following PS (50% vs. 29%,  = .001). Anemia, jaundice, and fatigue recurred in six patients with PS, leading to five completion splenectomies. Hemoglobin was not different between PS and TS by 5 years post-procedure (12.3 vs. 13.4 g/dL,  = .25). Both PS and TS ameliorate symptoms and improve hematologic parameters. The rate of secondary surgery following PS should be considered when planning the initial surgical procedure.

摘要

本研究比较了遗传性球形红细胞增多症患者行全脾切除术(TS)或部分脾切除术(PS)后的结局。共纳入79例患者(TS组33例,PS组46例)。PS组的随访期更长(59.6个月 vs. 24.9个月,P<0.001)。PS后长期不良事件的发生频率更高(50% vs. 29%,P = 0.001)。6例PS患者出现贫血、黄疸和疲劳复发,导致5例患者最终接受了全脾切除术。术后5年时,PS组和TS组的血红蛋白水平无差异(12.3 g/dL vs. 13.4 g/dL,P = 0.25)。PS和TS均能改善症状并改善血液学参数。在规划初始手术时,应考虑PS后的二次手术率。

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本文引用的文献

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Abnormalities of the erythrocyte membrane.红细胞膜异常。
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