Iron deficiency and anemia in patients with inherited bleeding disorders

VanderMeulen, Heather; Sholzberg, Michelle

doi:10.1016/j.transci.2018.10.015

Cited by 18 publications

(12 citation statements)

References 37 publications

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“…Despite the biological importance, the excess (hyperferremia) and the deficiency (hypoferremia) of iron can lead to serious health problems. The iron overload in the human body can cause severe diseases like osteoporosis, cancers, dysfunction of organs, hemochromatosis, and Alzheimer’s and Parkinson’s disease [2], whereas the iron deficiency can cause anemia and affect several cellular metabolic processes [3]. During the iron disorder, the labile iron generates destructive oxygen species (such as hydroxyl radical) via the Fenton reaction due to the facile redox process between Fe 2+ and Fe 3+ in the presence of molecular oxygen.…”

Section: Introductionmentioning

confidence: 99%

Recent Advances on Iron(III) Selective Fluorescent Probes with Possible Applications in Bioimaging

2019

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Iron(III) is well-known to play a vital role in a variety of metabolic processes in almost all living systems, including the human body. However, the excess or deficiency of Fe3+ from the normal permissible limit can cause serious health problems. Therefore, novel analytical methods are developed for the simple, direct, and cost-effective monitoring of Fe3+ concentration in various environmental and biological samples. Because of the high selectivity and sensitivity, fast response time, and simplicity, the fluorescent-based molecular probes have been developed extensively in the past few decades to detect Fe3+. This review was narrated to summarize the Fe3+-selective fluorescent probes that show fluorescence enhancement (turn-on) and ratiometric response. The Fe3+ sensing ability, mechanisms along with the analytical novelties of recently reported 77 fluorescent probes are discussed.

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Section: Introductionmentioning

confidence: 99%

Recent Advances on Iron(III) Selective Fluorescent Probes with Possible Applications in Bioimaging

2019

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“…In the pediatric population, IDA has been implicated in reduced stature, reduced growth velocity, altered immune function, and impaired cognitive function 19–23 . Rates of iron deficiency with and without anemia and the impact of IDA on bleeding management in BSS and GT are poorly characterized 24 . The ideal frequency of monitoring and therapeutic approach to iron deficiency in this population is also unclear 25 .…”

Section: Introductionmentioning

confidence: 99%

“…[19][20][21][22][23] Rates of iron deficiency with and without anemia and the impact of IDA on bleeding management in BSS and GT are poorly characterized. 24 The ideal frequency of monitoring and therapeutic approach to iron deficiency in this population is also unclear. 25 While limited case reports describe the use of short-term prophylactic rFVIIa as a method to prevent bleeding events not controlled by other therapies, 26 a standardized indication for long-term prophylactic rFVIIa has not been defined.…”

Section: Introductionmentioning

confidence: 99%

Iron deficiency anemia and bleeding management in pediatric patients with Bernard‐Soulier syndrome and Glanzmann Thrombasthenia: A single‐institution analysis

2022

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Introduction:Frequent and severe bleeding events (SBE) in patients with inherited qualitative platelet disorders Bernard-Soulier Syndrome (BSS) and Glanzmann Thrombasthenia (GT) can lead to secondary iron deficiency anemia (IDA). SBE are primarily treated with platelet transfusions or recombinant activated factor VII (rFVIIa) infusions. The impact of IDA on bleeding management and disease outcomes is understudied.Aim: To evaluate bleeding management, outcomes, and any association with IDA in pediatric patients with BSS and GT.Methods: Retrospective chart-review of pediatric patients with BSS or GT followed at a single hemophilia treatment center between 2007 and 2019. Results:We identified 14 patients with BSS (n = 2) or GT (n = 12). Patients received rFVIIa (7%), platelet transfusions (7%), or a combination of both (57%) for SBE.Eleven patients (79%) had IDA requiring oral and/or intravenous iron replacement and 50% required red blood cell transfusions. Due to recurrent SBE and refractory IDA, three patients (21%) received rFVIIa prophylaxis at 90 μg/kilogram 2-3 times/week for ≥15 months. Patients initiated on rFVIIa prophylaxis had a median baseline hemoglobin of 9.8 g/dL (min-max: 8.0-10.7 g/dL) compared to 11.7 g/dL (8.4-13.8 g/dL) for patients treated on-demand. Following initiation of rFVIIa prophylaxis, median hemoglobin and ferritin increased by 1.3 g/dL (0.7-2.5 g/dL) and 14.6 ng/mL (0.2-42.9 ng/mL), respectively, and bleeding rates were reduced by 7-78%. Conclusion:IDA is a known complication of recurrent bleeding events in individuals with inherited bleeding disorders. Routine monitoring for IDA may help improve bleeding management and reduce bleed burden in BSS/GT.

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“…[6][7][8] Menstruating adolescents, especially those with an inherited BD, are at increased risk of developing iron deficiency. 9 Iron deficiency can develop well before overt anemia presents. Even in the absence of anemia, iron deficiency can impair life quality.…”

Section: Introductionmentioning

confidence: 99%

Iron deficiency and fatigue among adolescents with bleeding disorders

et al. 2021

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Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is associated with a BD in adolescents. Moreover, the threshold of ferritin associated with fatigue in adolescents with HMB is unclear. In this multicenter study, we enrolled adolescents with HMB without BD. Participants underwent BD and anemia work-up in Young Women's Hematology Clinics and completed the Peds QL™ fatigue scale. BDs were defined as von Willebrand Disease, platelet function defect, clotting factor deficiencies, and hypermobility syndrome. Two hundred and fifty consecutive adolescents were enrolled, of whom 196 met eligibility criteria. Overall, 43% (95% confidence interval: 36%-50%) were diagnosed with BD. A total of 61% (n = 119) had serum ferritin levels < 15 ng/mL, 23.5% (n = 46) had iron deficiency only, and 37% (n = 73) had iron deficiency anemia. Low ferritin or ferritin dichoto-

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Iron deficiency and anemia in patients with inherited bleeding disorders

Cited by 18 publications

References 37 publications

Recent Advances on Iron(III) Selective Fluorescent Probes with Possible Applications in Bioimaging

Recent Advances on Iron(III) Selective Fluorescent Probes with Possible Applications in Bioimaging

Iron deficiency anemia and bleeding management in pediatric patients with Bernard‐Soulier syndrome and Glanzmann Thrombasthenia: A single‐institution analysis

Iron deficiency and fatigue among adolescents with bleeding disorders

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