Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis
[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...
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Institute of Blood Transfusion of Chinese Academy of Medical Sciences
2025-07-01
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| Series: | Zhongguo shuxue zazhi |
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| Online Access: | https://www.cjbt.cn/thesisDetails#10.13303/j.cjbt.issn.1004-549x.2025.07.008&lang=en |
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| author | LIU Haoqiang ZHAO Caihan YUAN Qing XIE Lixia ZOU Yong LU Ying |
| author_facet | LIU Haoqiang ZHAO Caihan YUAN Qing XIE Lixia ZOU Yong LU Ying |
| author_sort | LIU Haoqiang |
| collection | DOAJ |
| description | [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. |
| format | Article |
| id | doaj-art-b6a2b447ccf449e3aea702dd8ec27a51 |
| institution | DOAJ |
| issn | 1004-549X |
| language | zho |
| publishDate | 2025-07-01 |
| publisher | Institute of Blood Transfusion of Chinese Academy of Medical Sciences |
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| series | Zhongguo shuxue zazhi |
| spelling | doaj-art-b6a2b447ccf449e3aea702dd8ec27a512025-08-20T02:59:19ZzhoInstitute of Blood Transfusion of Chinese Academy of Medical SciencesZhongguo shuxue zazhi1004-549X2025-07-0138791592110.13303/j.cjbt.issn.1004-549x.2025.07.0081004-549X(2025)7-0915-07Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosisLIU Haoqiang0ZHAO Caihan1YUAN Qing2XIE Lixia3ZOU Yong4LU Ying5Department of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, ChinaDepartment of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, ChinaDepartment of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, ChinaDepartment of Blood Transfusion, Zhaoqing Hospital, the Third Affiliated Hospital of Sun Yat-sen University, Zhaoqing 526000, ChinaDepartment of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, ChinaDepartment of Blood Transfusion, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China[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.https://www.cjbt.cn/thesisDetails#10.13303/j.cjbt.issn.1004-549x.2025.07.008&lang=enrbc exchangeerythrocytapheresiserythropoietic protoporphyriahereditary hemochromatosis |
| spellingShingle | LIU Haoqiang ZHAO Caihan YUAN Qing XIE Lixia ZOU Yong LU Ying Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis Zhongguo shuxue zazhi rbc exchange erythrocytapheresis erythropoietic protoporphyria hereditary hemochromatosis |
| title | Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| title_full | Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| title_fullStr | Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| title_full_unstemmed | Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| title_short | Application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| title_sort | application and clinical efficacy of red blood cell therapeutic apheresis in erythropoietic protoporphyria and hereditary hemochromatosis |
| topic | rbc exchange erythrocytapheresis erythropoietic protoporphyria hereditary hemochromatosis |
| url | https://www.cjbt.cn/thesisDetails#10.13303/j.cjbt.issn.1004-549x.2025.07.008&lang=en |
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