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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Autor*in:	Marco Marziali [verfasserIn] Antonella Isgrò [verfasserIn] Pietro Sodani [verfasserIn] Javid Gaziev [verfasserIn] Daniela Fraboni [verfasserIn] Katia Paciaroni [verfasserIn] Cristiano Gallucci [verfasserIn] Cecilia Alfieri [verfasserIn] Andrea Roveda [verfasserIn] Gioia De Angelis [verfasserIn] Luisa Cardarelli [verfasserIn] Michela Ribersani [verfasserIn] Marco Andreani [verfasserIn] Guido Lucarelli [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2014

Schlagwörter:	Sickle Cell Disease Split Chimeirsm Bone Marrow

Übergeordnetes Werk:	In: Mediterranean Journal of Hematology and Infectious Diseases - Mattioli1885, 2010, 6(2014), 1, Seite e2014066-e2014066
Übergeordnetes Werk:	volume:6 ; year:2014 ; number:1 ; pages:e2014066-e2014066

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.4084/mjhid.2014.066

Katalog-ID:	DOAJ015706168

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callnumber-first	R - Medicine
author	Marco Marziali
spellingShingle	Marco Marziali misc RC633-647.5 misc Sickle Cell Disease misc Split Chimeirsm misc Bone Marrow misc Diseases of the blood and blood-forming organs 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
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topic_title	RC633-647.5 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 Sickle Cell Disease Split Chimeirsm Bone Marrow
topic	misc RC633-647.5 misc Sickle Cell Disease misc Split Chimeirsm misc Bone Marrow misc Diseases of the blood and blood-forming organs
topic_unstemmed	misc RC633-647.5 misc Sickle Cell Disease misc Split Chimeirsm misc Bone Marrow misc Diseases of the blood and blood-forming organs
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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
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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
author_sort	Marco Marziali
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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
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title_auth	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
abstract	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.
abstractGer	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.
abstract_unstemmed	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.
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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
url	https://doi.org/10.4084/mjhid.2014.066 https://doaj.org/article/e65dccd780f84903ad8c4c54612fc422 http://www.mjhid.org/index.php/mjhid/article/view/2056 https://doaj.org/toc/2035-3006
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up_date	2024-07-03T16:33:58.637Z
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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. 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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

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