一名接受血液透析的儿科患者中对促红细胞生成素刺激剂耐药的维生素B缺乏性贫血

Erythropoietin-stimulating agent-resistant vitamin B deficiency anemia in a pediatric patient on hemodialysis.

作者信息

Searcy Kristie, Rainwater Sarah, Jeroudi Majed, Baliga Radhakrishna

机构信息

LSU Health Sciences Center Shreveport, Shreveport, LA, USA.

LSU Health Sciences Center Shreveport, Section of Hematology-Oncology, Department of Pediatrics, Shreveport, LA, USA.

出版信息

Pediatr Nephrol. 2021 Feb;36(2):473-476. doi: 10.1007/s00467-020-04810-1. Epub 2020 Nov 6.

DOI:10.1007/s00467-020-04810-1

PMID:33156411

Abstract

BACKGROUND

Vitamin B 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] μm, corrected reticulocytes count 2.3% [0.2-1.8%], and vitamin B 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 B (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 B deficiency anemia should be considered in any pediatric patient on hemodialysis who does not respond to standard ESA and iron therapy.

摘要

背景

维生素B是一种限速辅酶，在血红素的生物合成以及铁整合到原卟啉的过程中发挥重要作用。其缺乏在慢性肾脏病（CKD）中经常可见，尤其是那些需要透析以及在使用促红细胞生成素刺激剂（ESA）之后的患者。

病例诊断/治疗：一名16岁非裔美国男性，患有5期CKD并接受慢性血液透析，在接受ESA治疗期间，血红蛋白在3个月内从11g/dl降至6.5g/dl，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）为87[78 - 98]μm，校正网织红细胞计数为2.3%[0.2 - 1.8%]，维生素B为1.2[5.3 - 46.7]μg/L。骨髓活检显示细胞正常（65%），有红系增生且罕见的红细胞生成异常。普鲁士蓝染色显示铁储存增加。在血红蛋白为7.3g/dL时开始补充维生素B（每日100mg），贫血得到纠正。18个月后，他的血红蛋白为11.7g/dL，转铁蛋白饱和度为45%，未再进行额外输血。