Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis
10.13303/j.cjbt.issn.1004-549x.2025.07.008
- VernacularTitle:红细胞相关治疗性单采在红细胞生成性卟啉病及遗传性血色病中的应用及临床疗效
- Author:
Haoqiang LIU
1
;
Caihan ZHAO
1
;
Qing YUAN
1
;
Lixia XIE
2
;
Yong ZOU
1
,
3
;
Ying LU
1
,
3
,
4
Author Information
1. Department of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China
2. Department of Blood Transfusion, Zhaoqing Hospital, the Third Affiliated Hospital of Sun Yat-sen University, Zhaoqing 526000, China
3. Department of Blood Transfusion, Zhaoqing Hospital, the Third Affiliated Hospital of Sun Yat-sen University, Zhaoqing 526000, China
4. The People's Hospital of Pome County, Pome 860399, China
- Publication Type:Journal Article
- Keywords:
RBC exchange;
erythrocytapheresis;
erythropoietic protoporphyria;
hereditary hemochromatosis
- From:
Chinese Journal of Blood Transfusion
2025;38(7):915-921
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To explore the application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria (EPP) and hereditary hemochromatosis (HH). Methods: 1) The EPP patient was hospitalized twice for "abdominal pain, nausea, vomiting, and brown urine". One and two sessions of red blood cell exchange/therapeutic plasma exchange (RCE/TPE) were respectively performed during the two hospitalizations. During each session, one RCE with 6-8 units of leukoreduced RBCs and 3-4 TPE procedures with 1 800-2 000 mL of frozen plasma was conducted. Biochemical parameters were monitored before and after treatment. 2) The HH patient was hospitalized for “repeatedly elevated aminotransferases”. Erythrocytapheresis was performed once, removing 550 mL of red blood cells, and venous phlebotomy was conducted once every 2 months subsequently. Blood routine and ferritin levels were assessed before and after treatment. Results: 1) During the first hospitalization, the EPP patient was relieved of the abdominal pain and brown urine after therapeutic apheresis. The total bilirubin level decreased from 141.8 μmol/L on admission to 68.6 μmol/L at discharge, with a symptom remission duration of 10 months. During the second hospitalization, the EPP patient still had recurrent abdominal pain after therapeutic apheresis. He developed psychiatric symptoms and gastrointestinal bleeding subsequently, accompanied by elevated bilirubin levels. Liver function deteriorated and the patient went into the state of the end-stage liver disease (ESLD). 2) For the HH patient, the hemoglobin level prior to erythrocytapheresis and vein phlebotomy was 150-160 g/L, with the lowest value occurring two days after erythrocytapheresis, decreasing to 107 g/L. The ferritin level before erythrocytapheresis was 2 428.08 ng/mL and it declined gradually after theraphy, with the lowest value occurring two months after erythrocytapheresis, decreasing to 1 094 ng/mL. The ferritin level was 1 114 ng/mL two months following the first vein phlebotomy, however it increased to 1 472 ng/mL two months after the second vein phlebotomy. Conclusion: RCE/TPE may alleviate protoporphyrin liver disease and help patients with bridging liver transplantation before EPP developments to ESLD. For HH patients with significantly elevated ferritin levels, erythrocytapheresis reduces serum ferritin more quickly and maintains its level longer relative to phlebotomy.
