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血液系统恶性肿瘤和骨髓衰竭综合征患儿输血相关铁过载(trio)的研究。

Study of transfusion-related iron overload (trio) in pediatric patients with hematological malignancy and bone marrow failure syndromes.

作者信息

Das Samannay, Misra Aroonima, Kashyap Archana, Meena Satish, Singh Amitabh, Aggarwal Kailash C

机构信息

Department of Pediatrics, VMMC and Safdarjung Hospital New Delhi, India.

ICMR-National Institute of Pathology, Safdarjung Hospital Campus New Delhi, India.

出版信息

Am J Blood Res. 2021 Aug 15;11(4):384-390. eCollection 2021.

Abstract

BACKGROUND

Pediatric patients with hematological malignancy and bone marrow failure syndrome receive multiple transfusions before diagnosis and treatment. Iron overload leads to damage to vital organs like the heart, liver, thyroid, Gonads, Pancreas.

MATERIALS AND METHODS

A prospective study was done from June 2017-December 2019 in a tertiary care pediatric hematology oncology unit in northern India on children diagnosed with hematological malignancy and bone marrow failure syndromes receiving packed cell transfusion. After due ethical considerations and patient consent, the details were documented in predesigned proforma. All cases were planned to be investigated with Liver function test, Thyroid function test, Serum ferritin level, 2 D Echocardiography, Ultrasonography of abdomen, and MRI of the abdomen at admission and six months of enrollment.

RESULTS

Out of 58 cases enrolled, ferritin levels were high in 65% of subjects at the start of treatment and 76% at the endpoint. Mean ferritin level was 725 ng/ml at baseline and 1268 ng/ml end of 6 month follow up period. Fifty-seven percent had a ferritin level above 1000 ng/ml, which correlated to basal ferritin level (-value 0.005). The final ferritin level correlated strongly with the final number of packed cell transfusions (-value 0.0002). Functional derangement of the liver was evident biochemically in 13.7% before starting treatment and 31.8% at six months follow-up period. Echocardiography detected diastolic dysfunction in 2% of patients at baseline before starting treatment and increased to 22% in 6 months follow-up period. The percentage of subclinical hypothyroidism increased from 22.8% to 48.8% during treatment.

CONCLUSION

Like transfusion-dependent anemias, children with hematological malignancy and bone marrow failure syndrome on chronic transfusion are at risk of transfusion-related iron overload and organ damage.

摘要

背景

患有血液系统恶性肿瘤和骨髓衰竭综合征的儿科患者在诊断和治疗前接受多次输血。铁过载会导致心脏、肝脏、甲状腺、性腺、胰腺等重要器官受损。

材料与方法

2017年6月至2019年12月在印度北部一家三级儿科血液肿瘤病房对诊断为血液系统恶性肿瘤和骨髓衰竭综合征且接受红细胞悬液输血的儿童进行了一项前瞻性研究。经过适当的伦理考量和患者同意后,将详细信息记录在预先设计的表格中。所有病例计划在入院时和入组6个月时进行肝功能检查、甲状腺功能检查、血清铁蛋白水平检测、二维超声心动图检查、腹部超声检查以及腹部磁共振成像检查。

结果

在纳入的58例病例中,65%的受试者在治疗开始时铁蛋白水平较高,在终点时这一比例为76%。基线时平均铁蛋白水平为725 ng/ml,6个月随访期结束时为1268 ng/ml。57%的患者铁蛋白水平高于1000 ng/ml,这与基础铁蛋白水平相关(P值为0.005)。最终铁蛋白水平与红细胞悬液输血的最终次数密切相关(P值为0.0002)。治疗开始前,13.7%的患者在生化方面表现出明显的肝功能紊乱,在6个月随访期时这一比例升至31.8%。超声心动图检查发现,治疗开始前基线时2%的患者存在舒张功能障碍,在6个月随访期时增至22%。亚临床甲状腺功能减退的比例在治疗期间从22.8%增至48.8%。

结论

与输血依赖型贫血一样,患有血液系统恶性肿瘤和骨髓衰竭综合征且长期输血的儿童有输血相关铁过载和器官损伤的风险。

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