Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease
Allogeneic cellular gene therapy through hematopoietic stem cell transplantation is the only radical cure for congenital hemoglobinopathies like thalassemia and sickle cell anemia. Persistent mixed hematopoietic chimerism (PMC) has been described in thalassemia and sickle cell anemia. Here, we descr...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2014-08-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | http://www.mjhid.org/index.php/mjhid/article/view/2056 |
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| author | Marco Marziali Antonella Isgrò Pietro Sodani Javid Gaziev Daniela Fraboni Katia Paciaroni Cristiano Gallucci Cecilia Alfieri Andrea Roveda Gioia De Angelis Luisa Cardarelli Michela Ribersani Marco Andreani Guido Lucarelli |
| author_facet | Marco Marziali Antonella Isgrò Pietro Sodani Javid Gaziev Daniela Fraboni Katia Paciaroni Cristiano Gallucci Cecilia Alfieri Andrea Roveda Gioia De Angelis Luisa Cardarelli Michela Ribersani Marco Andreani Guido Lucarelli |
| author_sort | Marco Marziali |
| collection | DOAJ |
| description | Allogeneic cellular gene therapy through hematopoietic stem cell transplantation is the only radical cure for congenital hemoglobinopathies like thalassemia and sickle cell anemia. Persistent mixed hematopoietic chimerism (PMC) has been described in thalassemia and sickle cell anemia. Here, we describe the clinical course of a 6-year-old girl who had received bone marrow transplant for sickle cell anemia. After the transplant, the patient showed 36% donor hematopoietic stem cells in the bone marrow, whereas in the peripheral blood there was evidence of 80% circulating donor red blood cells (RBC). The analysis of apoptosis at the Bone Marrow level suggests that Fas might contribute to the cell death of host erythroid precursors. The increase in NK cells and the regulatory T cell population observed in this patient suggests that these cells might contribute to the condition of mixed chimerism. |
| format | Article |
| id | doaj-art-e65dccd780f84903ad8c4c54612fc422 |
| institution | DOAJ |
| issn | 2035-3006 |
| language | English |
| publishDate | 2014-08-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-e65dccd780f84903ad8c4c54612fc4222025-08-20T02:58:04ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062014-08-0161e2014066e201406610.4084/mjhid.2014.0661512Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell DiseaseMarco Marziali0Antonella Isgrò1Pietro Sodani2Javid Gaziev3Daniela Fraboni4Katia Paciaroni5Cristiano Gallucci6Cecilia Alfieri7Andrea Roveda8Gioia De Angelis9Luisa Cardarelli10Michela Ribersani11Marco Andreani12Guido Lucarelli13International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyDepartment of Biopathology and Diagnostic Images, Polyclinic of Tor Vergata Foundation, Rome, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyLaboratory of Immunogenetics and Transplant Biology, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor VergataInternational Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, ItalyAllogeneic cellular gene therapy through hematopoietic stem cell transplantation is the only radical cure for congenital hemoglobinopathies like thalassemia and sickle cell anemia. Persistent mixed hematopoietic chimerism (PMC) has been described in thalassemia and sickle cell anemia. Here, we describe the clinical course of a 6-year-old girl who had received bone marrow transplant for sickle cell anemia. After the transplant, the patient showed 36% donor hematopoietic stem cells in the bone marrow, whereas in the peripheral blood there was evidence of 80% circulating donor red blood cells (RBC). The analysis of apoptosis at the Bone Marrow level suggests that Fas might contribute to the cell death of host erythroid precursors. The increase in NK cells and the regulatory T cell population observed in this patient suggests that these cells might contribute to the condition of mixed chimerism.http://www.mjhid.org/index.php/mjhid/article/view/2056Sickle Cell DiseaseSplit ChimeirsmBone Marrow |
| spellingShingle | Marco Marziali Antonella Isgrò Pietro Sodani Javid Gaziev Daniela Fraboni Katia Paciaroni Cristiano Gallucci Cecilia Alfieri Andrea Roveda Gioia De Angelis Luisa Cardarelli Michela Ribersani Marco Andreani Guido Lucarelli Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease Mediterranean Journal of Hematology and Infectious Diseases Sickle Cell Disease Split Chimeirsm Bone Marrow |
| title | Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease |
| title_full | Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease |
| title_fullStr | Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease |
| title_full_unstemmed | Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease |
| title_short | Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease |
| title_sort | peripheral red blood cell split chimerism as a consequence of intramedullary selective apoptosis of recipient red blood cells in a case of sickle cell disease |
| topic | Sickle Cell Disease Split Chimeirsm Bone Marrow |
| url | http://www.mjhid.org/index.php/mjhid/article/view/2056 |
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