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输血依赖型地中海贫血脾切除术的长期结果

Long-term Results of Splenectomy in Transfusion-dependent Thalassemia.

作者信息

Akca Tugberk, Ozdemir Gul N, Aycicek Ali, Ozkaya Guven

机构信息

Departments of Pediatrics.

Departments of Pediatric Cardiology.

出版信息

J Pediatr Hematol Oncol. 2023 Apr 1;45(3):143-148. doi: 10.1097/MPH.0000000000002468. Epub 2022 Apr 18.

Abstract

Splenectomy is indicated in transfusion-dependent thalassemia (TDT) only in certain situations. This study aimed to present the effectiveness, complications, and long-term follow-up results of splenectomy in children with TDT. We performed a 30-year single-institution analysis of cases of splenectomy for TDT between 1987 and 2017 and their follow-up until 2021. A total of 39 children (female/male: 24/15) were included. The mean age at splenectomy was 11.2±3.2 years, and their mean follow-up duration after splenectomy was 21.5±6.4 years. Response was defined according to the patient's annual transfusion requirement in the first year postsplenectomy and on the last follow-up year. Complete response was not seen in any of the cases; partial response was observed in 32.3% and no response in 67.6%. Thrombocytosis was seen in 87% of the patients. The platelet counts of 7 (17.9%) patients were >1000 (10 9 /L), and aspirin prophylaxis was given to 22 (56.4%) patients. Complications were thrombosis in 2 (5.1%) patients, infections in 11 (28.2%) patients, and pulmonary hypertension in 4 (10.2%) patients. Our study showed that after splenectomy, the need for transfusion only partially decreased in a small number of TDT patients. We think splenectomy can be delayed with appropriate chelation therapy up to higher annual transfusion requirement values.

摘要

脾切除术仅在某些情况下适用于依赖输血的地中海贫血(TDT)。本研究旨在介绍TDT患儿脾切除术的有效性、并发症及长期随访结果。我们对1987年至2017年间在单一机构进行的TDT脾切除术病例及其至2021年的随访进行了为期30年的分析。共纳入39名儿童(女/男:24/15)。脾切除时的平均年龄为11.2±3.2岁,脾切除术后的平均随访时间为21.5±6.4年。根据患者脾切除术后第一年及最后随访年的年度输血需求来定义反应。所有病例均未出现完全反应;32.3%观察到部分反应,67.6%无反应。87%的患者出现血小板增多症。7名(17.9%)患者的血小板计数>1000(10⁹/L),22名(56.4%)患者给予了阿司匹林预防性治疗。并发症包括2名(5.1%)患者发生血栓形成,11名(28.2%)患者发生感染,4名(10.2%)患者发生肺动脉高压。我们的研究表明,脾切除术后,少数TDT患者的输血需求仅部分降低。我们认为,通过适当的螯合疗法,脾切除术可推迟至更高的年度输血需求值。

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