血液恶性肿瘤和骨髓衰竭综合征儿科患者输血相关铁过载(trio)研究。

American journal of blood research Pub Date : 2021-08-15 eCollection Date: 2021-01-01
Samannay Das, Aroonima Misra, Archana Kashyap, Satish Meena, Amitabh Singh, Kailash C Aggarwal
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引用次数: 0

摘要

背景:儿童血液恶性肿瘤和骨髓衰竭综合征患者在诊断和治疗前会接受多次输血。铁超载会导致心脏、肝脏、甲状腺、性腺、胰腺等重要器官受损:一项前瞻性研究于 2017 年 6 月至 2019 年 12 月在印度北部的一家三级医疗机构儿科血液肿瘤科进行,研究对象是确诊为血液恶性肿瘤和骨髓衰竭综合征并接受填料细胞输血的儿童。在充分考虑伦理因素并征得患者同意后,在预先设计的表格中记录了详细情况。所有病例均计划在入院和入院 6 个月时接受肝功能检查、甲状腺功能检查、血清铁蛋白水平、2D 超声心动图、腹部超声波检查和腹部核磁共振成像检查:在 58 例入组病例中,65% 的受试者在治疗开始时铁蛋白水平较高,76% 的受试者在治疗结束时铁蛋白水平较高。基线铁蛋白平均水平为 725 纳克/毫升,6 个月随访结束时为 1268 纳克/毫升。57%的受试者铁蛋白水平高于 1000 纳克/毫升,这与基础铁蛋白水平相关(P 值为 0.005)。最终的铁蛋白水平与最终的包装细胞输血次数密切相关(P 值为 0.0002)。13.7% 的患者在开始治疗前就出现了明显的生化肝功能异常,31.8% 的患者在随访 6 个月后出现了明显的生化肝功能异常。超声心动图检查发现,开始治疗前有 2% 的患者存在舒张功能障碍,随访 6 个月后这一比例增至 22%。亚临床甲状腺功能减退症的比例在治疗期间从22.8%增至48.8%:与输血依赖性贫血一样,长期输血的血液恶性肿瘤和骨髓衰竭综合征患儿也面临输血相关铁过载和器官损伤的风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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

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

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

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 (P-value 0.005). The final ferritin level correlated strongly with the final number of packed cell transfusions (P-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.

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American journal of blood research
American journal of blood research MEDICINE, RESEARCH & EXPERIMENTAL-
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