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Background: Synthesis of hemoglobin is one of several important roles iron plays in the human body. Approximately 50% of all anemia cases may be caused by iron deficiency which is frequently caused by chronic blood loss. Patients with bleeding disorders have greater propensity for blood loss and therefore may have a higher prevalence of iron deficiency when compared to the general population. However, few studies have assessed the prevalence of iron deficiency in children with inherited bleeding disorders. Objectives: This study aims to identify the prevalence of iron deficiency in children with an inherited bleeding disorder. Methods: A retrospective analysis of children with any inherited bleeding disorder seen in Children’s Mercy Hospital’s hemophilia treatment center between 2010 and 2020 was performed. Iron deficiency was defined by recently published serum ferritin thresholds outlined by the National Health and Nutrition Examination Surveys (NHANES) [1]. Subjects were defined as iron deficient if they met these criteria at any visit. Other patient characteristics such as concomitant iron deficiency risk factors and use of iron supplementation were included in the analysis. Results: There were 507 patients with inherited bleeding disorders who were included in this analysis. Of the 177 patients who had serum ferritin collected, 103 (58%) were iron deficient. In patients who were iron deficient, 69% were female and 51% were aged 6 to 15 years. Notably, of the 68 (38%) males with serum ferritin collected, 31% were iron deficient. Of the 478 patients who had blood counts measured, 217 (45%) were anemic. Of the patients with anemia, 138 (64%) were male. The proportion of patients found to be iron deficient in each age group are displayed in figure 1. Only 4 (4%) of the patients found to be iron deficient had other iron deficiency risk factors. Furthermore, 64 (62%) of patients who were identified as iron deficient were on some form of iron supplementation. Conclusion: The results of this study suggest that iron deficiency is more prevalent in patients with inherited bleeding disorders when compared to the general population. Prior epidemiologic analysis of iron deficiency and iron deficiency anemia is mostly focused on adolescent females, but these results suggest that males with inherited bleeding disorders are at comparable risk. The actual prevalence of iron deficiency in male patients may be higher than this report suggests considering ferritin levels were not measured in 75% of males. Furthermore, the prevalence of iron deficiency is likely underreported in studies prior to the recently published NHANES physiologic ferritin thresholds. These patients represent half of the population identified for inclusion in this retrospective study.


Hematology | Pediatrics


Presented at the Hemophilia and Thrombosis Research Symposium (HTRS) Scientific Symposium; Orlando, Florida; March 10-12, 2023.

Prevalence of Iron Deficiency in Patients with Inherited Bleeding Disorders