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

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
  • Marco Marziali
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy | m.marziali@fondazioneime.org
  • Antonella Isgrò
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Pietro Sodani
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Javid Gaziev
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Daniela Fraboni
    Department of Biopathology and Diagnostic Images, Polyclinic of Tor Vergata Foundation, Rome, Italy, Italy
  • Katia Paciaroni
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Cristiano Gallucci
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Cecilia Alfieri
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Andrea Roveda
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Gioia De Angelis
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Luisa Cardarelli
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Michela Ribersani
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy
  • Marco Andreani
    Laboratory of Immunogenetics and Transplant Biology, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy
  • Guido Lucarelli
    International Center for Transplantation in Thalassemia and Sickle Cell Anemia, Mediterranean Institute of Hematology, Policlinic of the University of Rome “Tor Vergata, Italy, Italy

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.

Keywords

Sickle Cell Disease; Split Chimeirsm; Bone Marrow

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Submitted: 2014-08-29 14:25:24
Published: 2014-08-28 00:00:00
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