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.

中文翻译：

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儿童血液透析患者促红细胞生成素刺激剂抗性维生素 B 6 缺乏性贫血

背景

维生素 B ₆是一种限速辅酶，在血红素的生物合成和铁与原卟啉的结合中起重要作用。其缺乏常见于慢性肾病 (CKD)，尤其是那些需要透析和服用促红细胞生成素刺激剂 (ESA) 的患者。

病例-诊断/治疗

一名患有慢性血液透析 5 期 CKD 的 16 岁非洲裔美国男性在接受 ESA 治疗期间的 3 个月内血红蛋白从 11 克/分升降至 6.5 克/分升，导致多次输血。他的转铁蛋白饱和度为 41%，铁蛋白水平为 706 [80-388] ng/mL，平均红细胞体积 (MCV) 87 [78-98] μm ³，校正网织红细胞计数为 2.3% [0.2-1.8%]，以及维生素 B ₆ 1.2 [5.3–46.7] μg/L。骨髓活检显示细胞正常（65%），伴有红细胞增生和罕见的红细胞生成异常。普鲁士蓝染色显示铁储存增加。补充维生素 B ₆（每天 100 毫克）以血红蛋白 7.3 g/dL 开始，并纠正贫血。18 个月后，他的血红蛋白为 11.7 g/dL，转铁蛋白饱和度为 45%，没有额外输血。

结论

任何对标准 ESA 和铁治疗无反应的血液透析儿科患者都应考虑维生素 B ₆缺乏性贫血。