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Hematology Reports
Volume 12
Issue 1
10.4081/hr.2020.8305
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Successful Allogeneic Bone Marrow Transplantation Using Immunosuppressive Conditioning Regimen for a Patient with Red Blood Cell Transfusion-Dependent Pyruvate Kinase Deficiency Anemia

by
Maiko Shimomura
*,
Takehiko Doi
,
Shiho Nishimura
,
Yusuke Imanaka
,
Shuhei Karakawa
,
Satoshi Okada
,
Hiroshi Kawaguchi
and
Masao Kobayashi
Department of Pediatrics, Hiroshima University Graduate School of Biomedical anda Health Sciences, 1-2-3 Kasumi, Minamiku, Hiroshima City, Hiroshima 734-8553 Japan
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2020, 12(1), 8305; https://doi.org/10.4081/hr.2020.8305
Submission received: 12 September 2019 / Revised: 25 November 2019 / Accepted: 31 January 2020 / Published: 15 May 2020

Abstract

Pyruvate kinase deficiency (PKD) is the rare glycolytic enzyme defect causing hemolytic anemia. Treatments are mainly red cell transfusion and/or splenectomy, leading to iron overload. Allogeneic bone marrow transplantation (BMT) is alternatively curative treatment for severe PKD. The intensity of conditioning is often controversial because of higher risk of graft failure and organ damage. Here, we present a transfusion-dependent PKD patient undergoing BMT from an HLA-identical sibling using intensively immunosuppressive conditioning regimen. This report suggests that BMT using immunosuppressive conditioning regimen may be a feasible and effective treatment for patients with severe PKD with iron overload. We suggest the timing of the transplantation at an earlier age in severe PKD predicted from gene mutation is preferred before cumulative damage of transfusion.
Keywords: pyruvate kinase deficiency; allogeneic hematopoietic cell transplantation; immunosuppressive conditioning regimen pyruvate kinase deficiency; allogeneic hematopoietic cell transplantation; immunosuppressive conditioning regimen

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MDPI and ACS Style

Shimomura, M.; Doi, T.; Nishimura, S.; Imanaka, Y.; Karakawa, S.; Okada, S.; Kawaguchi, H.; Kobayashi, M. Successful Allogeneic Bone Marrow Transplantation Using Immunosuppressive Conditioning Regimen for a Patient with Red Blood Cell Transfusion-Dependent Pyruvate Kinase Deficiency Anemia. Hematol. Rep. 2020, 12, 8305. https://doi.org/10.4081/hr.2020.8305

AMA Style

Shimomura M, Doi T, Nishimura S, Imanaka Y, Karakawa S, Okada S, Kawaguchi H, Kobayashi M. Successful Allogeneic Bone Marrow Transplantation Using Immunosuppressive Conditioning Regimen for a Patient with Red Blood Cell Transfusion-Dependent Pyruvate Kinase Deficiency Anemia. Hematology Reports. 2020; 12(1):8305. https://doi.org/10.4081/hr.2020.8305

Chicago/Turabian Style

Shimomura, Maiko, Takehiko Doi, Shiho Nishimura, Yusuke Imanaka, Shuhei Karakawa, Satoshi Okada, Hiroshi Kawaguchi, and Masao Kobayashi. 2020. "Successful Allogeneic Bone Marrow Transplantation Using Immunosuppressive Conditioning Regimen for a Patient with Red Blood Cell Transfusion-Dependent Pyruvate Kinase Deficiency Anemia" Hematology Reports 12, no. 1: 8305. https://doi.org/10.4081/hr.2020.8305

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