Erythropoietin-stimulating agent-resistant vitamin B 6 deficiency anemia in a pediatric patient on hemodialysis
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BRIEF REPORT
Erythropoietin-stimulating agent-resistant vitamin B6 deficiency anemia in a pediatric patient on hemodialysis Kristie Searcy 1
&
Sarah Rainwater 1 & Majed Jeroudi 1,2 & Radhakrishna Baliga 1,3
Received: 20 July 2020 / Revised: 13 September 2020 / Accepted: 2 October 2020 # IPNA 2020
Abstract Background Vitamin B6 is a rate-limiting coenzyme that plays an important role in the biosynthesis of heme and the incorporation of iron into protoporphyrin. Its deficiency is often seen in chronic kidney disease (CKD), particularly those requiring dialysis and following administration of erythropoietin-stimulating agent (ESA). Case- diagnosis/treatment A 16-year-old African-American male with stage 5 CKD on chronic hemodialysis experienced a decrease in hemoglobin over a 3-month period from 11 to 6.5 g/dl while receiving ESA, resulting in multiple blood transfusions. His transferrin saturation was 41%, ferritin level 706 [80–388] ng/mL, mean corpuscular volume (MCV) 87 [78–98] μm3, corrected reticulocytes count 2.3% [0.2–1.8%], and vitamin B6 1.2 [5.3–46.7] μg/L. Bone marrow biopsy was normocellular (65%) with erythroid hyperplasia and rare dyserythropoiesis. Prussian blue staining showed increased iron storage. Supplemental vitamin B6 (100 mg daily) was initiated at hemoglobin 7.3 g/dL with correction of anemia. Eighteen months later, his hemoglobin is 11.7 g/dL, transferrin saturation 45%, with no additional blood transfusions. Conclusions Vitamin B6 deficiency anemia should be considered in any pediatric patient on hemodialysis who does not respond to standard ESA and iron therapy. Keywords Vitamin B6 . Anemia . Erythropoietin stimulating agent (ESA) . Chronic kidney disease . Kidney failure . High flux/ high efficiency (HF/HE) hemodialysis
Introduction Vitamin B6 is a water-soluble coenzyme, the biologically active form of which is the phosphate ester derivative pyridoxal 5′-phosphate (PLP) that serves as a cofactor in the formation of erythroid specific 5-aminolevulinate synthase (ALAS2) which is involved in the synthesis of heme [1]. It also plays a vital role in the incorporation of iron into protoporphyrin, the final step of heme synthesis [1]. Vitamin B6 deficiency is often associated with inflammation, as observed in conditions such as chronic kidney disease (CKD),
* Kristie Searcy [email protected]
particularly those requiring dialysis [2]. Erythropoietin deficiency is the clinical hallmark of progressive kidney disease, especially in patients receiving maintenance dialysis. Erythropoietin-stimulating agent (ESA) increases the rate of erythropoiesis and the demand for iron, thus increasing vitamin B6 consumption [3]. We report here a pediatric patient with stage 5 CKD on chronic maintenance hemodialysis who developed an ESA-resistant anemia that substantially improved following vitamin B6 supplementation. As far as we know, ours is the only pediatric patient on hemodialysis with ESA-resistant pyridoxine-responsive anemia.
1
LSU Health Sciences Center Shreveport, Shreveport, LA, USA
2
LSU He
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