Hemostatic abnormalities in children with thalassemia major and liver iron overload

Abstract

Background Thalassemia major (TM) patients are susceptible to liver dysfunction due to iron deposition. Pediatric TM patients often present with bleeding. Blood loss necessitates transfusions, leading to increased iron absorption from the gut.
 Objective To study hemostatic abnormalities in children with TM and iron deposition in the liver.
 Methods This cross-sectional study involved 190 non-splenectomized children with TM. Liver iron deposition was evaluated using T2* MRI. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet counts were assessed from blood specimens.
 Results Most subjects were diagnosed with β-thalassemia and β-thalassemia/HbE. The majority of subjects were on deferiprone (DFP) treatment. Approximately 89.5% of subjects had liver iron overload. Prolongation of PT and aPTT, as well as thrombocytopenia were observed in 60%, 27.9%, and 19.5% of subjects, respectively. Prolonged aPTT and thrombocytopenia were observed three times more frequently in subjects with moderate - severe liver iron overload than in subjects with normal - mild liver iron overload (P=0.04 and 0.001, respectively).
 Conclusion Most TM subjects have liver iron overload ranging from mild to severe. Prothrombin time and prolongation, as well as aPTT prolongation, and thrombocytopenia are easily found in TM children. There were significantly more moderate - severe liver iron deposition patients with aPTT prolongation and thrombocytopenia than normal – mild patients with these conditions. Hence, we suggest that pediatric TM patients undergo liver iron deposition evaluations and use iron chelators in an optimal manner, in order to limit the risk of bleeding.

Glucose and Lipid Profiles in Adolescents with Thalassemia Major and Its Association with Iron Overload in Specific Organs

“…(28,29) Liver iron overload in thalassemia major patients causes impairment on its function. (30,31) Theoretically, liver plays an important role in production, storage, and metabolism of lipid and lipoprotein in the body. One study finds that HDL-C and LDL-C levels are significantly affected by the severity of liver injury in non-alcoholic liver cirrhosis.…”

Association Between Immune-Inflammation Index (SII) and Ferritin with Hepatic Function in Patients with Thalassemia Major

“…While chronic hypercoagulability is widely observed in thalassemia patients, it is mainly reported in adult subjects. Furthermore, 29.6% of pediatric patients were reported to experience epistaxis and gum bleeding 28 .…”