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Αλέξανδρος Γ. Σφακιανάκης

Monday, September 20, 2021

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

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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.

RESUL TS: 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 transfusi on are at risk of transfusion-related iron overload and organ damage.

PMID:34540346 | PMC:PMC8446827

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