Expression and influencing factors of hepcidin in classical paroxysmal nocturnal hemoglobinuria
10.3760/cma.j.issn.0253-2727.2019.10.004
- VernacularTitle: 铁调素在经典型阵发性睡眠性血红蛋白尿症中的表达及影响因素分析
- Author:
Yansheng WANG
1
;
Hongjing YAO
;
Lijin BO
;
Yuping ZHAO
;
Yali ZHANG
;
Fengkui ZHANG
Author Information
1. Institute of Hematology and Blood Disease Hospital, CAMS & PUMC, National Clinical Research Center for Blood Diseases, Tianjin 300020, China
- Publication Type:Journal Article
- Keywords:
Hepcidin;
Paroxysmal nocturnal hemoglobinuria
- From:
Chinese Journal of Hematology
2019;40(10):818-821
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the serum expression and influencing factors of hepcidinin patients with classical paroxysmal nocturnal hemoglobinuria (PNH) .
Methods:Retrospective analysis of 36 classical PNH patients from 2016.3 to 2017.3. Serum hepcidin concentration was measured by ELISA method. The relationship between serum hepcidin concentration and erythropoiesis and iron homeostasis parameters was evaluated.
Results:The median serum hepcidin level of 36 classical PNH patients was 32.03 (23.11, 118.48) μg/L, it was significantly lower than of 181.42 (106.80, 250.53) μg/L in 292 normal control subjects (z=-5.107, P<0.001) . The median serum hepcidin of 56.41 (44.60, 95.06) μg/L in PNH patients with normal ferritin was significantly lower than that in normal controls. The median serum hepcidin concentration 23.75 (21.77, 30.35) μg/L in iron deficiency PNH patients was lower than that in the normal ferritin PNH patients. However, the median serum hepcidin level of classical PNH with elevated ferritin patients 336.19 (304.19, 375.08) μg/L was significantly higher not only than that of normal ferritin and iron deficiency PNH ones, but also than that of normal control subjects. Regression analysis showed that serum ferritin, transferrin saturation and serum albumin level were independent influencing factors of serum hepcidin level in patients with classical PNH.
Conclusion:The decreased serum hepcidin level in patients with classical PNH was mainly influenced by iron metabolism factors.