Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease

Abstract Hemoglobin H (HbH) disease is associated with anemia, ineffective erythropoiesis, and iron overload. We report a case of a patient with HbH/Hb Constant Spring disease, who was maintained on chronic transfusions as an adult due to symptomatic anemia. Over time, he developed iron overload and...

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Main Authors: Ke Zhang, John Bliamptis, Janice Park, Patricia Kopko, Amber P. Sanchez, Srila Gopal
Format: Article
Language:English
Published: Wiley 2025-02-01
Series:eJHaem
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Online Access:https://doi.org/10.1002/jha2.1089
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author Ke Zhang
John Bliamptis
Janice Park
Patricia Kopko
Amber P. Sanchez
Srila Gopal
author_facet Ke Zhang
John Bliamptis
Janice Park
Patricia Kopko
Amber P. Sanchez
Srila Gopal
author_sort Ke Zhang
collection DOAJ
description Abstract Hemoglobin H (HbH) disease is associated with anemia, ineffective erythropoiesis, and iron overload. We report a case of a patient with HbH/Hb Constant Spring disease, who was maintained on chronic transfusions as an adult due to symptomatic anemia. Over time, he developed iron overload and was started on chelation therapy but did not have an adequate response to chelation. We then added erythrocytapheresis to chelation therapy and were able to successfully decrease his iron burden while managing his anemia. Therapeutic erythrocytapheresis may be an effective treatment strategy for iron overload in HbH disease that is refractory to chelation.
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language English
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publisher Wiley
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series eJHaem
spelling doaj-art-41ec3d3e278047f7972972a274833ca52025-08-20T02:46:50ZengWileyeJHaem2688-61462025-02-0161n/an/a10.1002/jha2.1089Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H diseaseKe Zhang0John Bliamptis1Janice Park2Patricia Kopko3Amber P. Sanchez4Srila Gopal5Department of Internal Medicine Capital Medical University Beijing ChinaDivision of Internal Medicine University of California San Diego La Jolla California USADivision of Nephrology University of California San Diego La Jolla California USADivision of Pathology University of California San Diego La Jolla California USADivision of Nephrology University of California San Diego La Jolla California USADivision of Hematology University of California San Diego La Jolla California USAAbstract Hemoglobin H (HbH) disease is associated with anemia, ineffective erythropoiesis, and iron overload. We report a case of a patient with HbH/Hb Constant Spring disease, who was maintained on chronic transfusions as an adult due to symptomatic anemia. Over time, he developed iron overload and was started on chelation therapy but did not have an adequate response to chelation. We then added erythrocytapheresis to chelation therapy and were able to successfully decrease his iron burden while managing his anemia. Therapeutic erythrocytapheresis may be an effective treatment strategy for iron overload in HbH disease that is refractory to chelation.https://doi.org/10.1002/jha2.1089erythrocytapheresisiron overloadthalassemia
spellingShingle Ke Zhang
John Bliamptis
Janice Park
Patricia Kopko
Amber P. Sanchez
Srila Gopal
Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
eJHaem
erythrocytapheresis
iron overload
thalassemia
title Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
title_full Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
title_fullStr Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
title_full_unstemmed Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
title_short Erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin H disease
title_sort erythrocytapheresis as a strategy to manage anemia and iron overload in nondeletional hemoglobin h disease
topic erythrocytapheresis
iron overload
thalassemia
url https://doi.org/10.1002/jha2.1089
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AT johnbliamptis erythrocytapheresisasastrategytomanageanemiaandironoverloadinnondeletionalhemoglobinhdisease
AT janicepark erythrocytapheresisasastrategytomanageanemiaandironoverloadinnondeletionalhemoglobinhdisease
AT patriciakopko erythrocytapheresisasastrategytomanageanemiaandironoverloadinnondeletionalhemoglobinhdisease
AT amberpsanchez erythrocytapheresisasastrategytomanageanemiaandironoverloadinnondeletionalhemoglobinhdisease
AT srilagopal erythrocytapheresisasastrategytomanageanemiaandironoverloadinnondeletionalhemoglobinhdisease