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.
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.
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Marziali
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Isgrò
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Sodani
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Javid
authors.initials: J
authors.lastname: Gaziev
authors.affiliation: Department of Biopathology and Diagnostic Images, Polyclinic of Tor Vergata Foundation, Rome, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Fraboni
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Katia
authors.initials: K
authors.lastname: Paciaroni
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Cristiano
authors.initials: C
authors.lastname: Gallucci
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Alfieri
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Roveda
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Gioia
authors.initials: G
authors.lastname: De Angelis
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Cardarelli
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Ribersani
authors.affiliation: Laboratory of Immunogenetics and Transplant Biology, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata"
authors.forename: Marco
authors.initials: M
authors.lastname: Andreani
authors.affiliation: International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome "Tor Vergata" Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Lucarelli