We report here the reconstitution after bone marrow transplantation (BMT) in identical infant twins with acute myelogenous leukemia (AML). They were diagnosed at 8 and 9 months of age. Complete remission was induced after two courses of chemotherapy. After four and five courses of chemotherapy, respectively, they received BMT at 2-month interval from the same HLA-identical older brother. The total dose of marrow nucleated cells (NC) harvested was 77.7 x 10(8). The first patient was transplanted with half of the total dose of NC. The remaining cells were cryopreserved without the use of a programmed freezer and transplanted into the second patient 2 months later. The number of days for neutrophil (>0.5 x 10(9)/L), platelet (>50 x 10(9)/L), and reticulocyte (>1%) recovery were, respectively, 15, 21, and 14 in the first case and 12, 21, and 15 in the second case. The clinical courses after BMT were uneventful in both cases, except for mild acute GVHD, and complete remission has been maintained >4 yr with full recovery of immune and marrow function. Based on the results in these cases, we confirmed that marrow cells that have been cryopreserved without the use of a programmed freezer could reconstitute immune and marrow function as well as non-cryopreserved cells.
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http://dx.doi.org/10.1111/j.1399-0012.2004.00273.x | DOI Listing |
Immunol Cell Biol
January 2025
The Babraham Institute, Babraham Hall, Cambridge, UK.
This report presents findings from a group of UK-based researchers with expertise in the use of animal models for bone marrow ablation and reconstitution. The primary aim is to facilitate the implementation of the Three Rs (Replacement, Reduction and Refinement), with an emphasis on refinement. Bone marrow ablation and reconstitution procedures are performed for a number of different purposes and conducted predominantly in mice.
View Article and Find Full Text PDFEur Respir J
January 2025
Department of Biochemistry and Molecular Biology, Brain Korea 21 Project, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Rationale: Although a relationship between the Gas6/AXL pathway and pulmonary fibrosis (PF) has been suggested, the precise mechanisms and clinical implications of the AXL pathway in idiopathic pulmonary fibrosis (IPF) are still unclear.
Methods: Constitutive and conditional AXL-knockout mice were generated and injected with bleomycin (BLM) to induce pulmonary fibrosis. The expression of AXL and macrophage subtypes in BLM-injected mice and patients with IPF was analysed using flow cytometry.
JAGN1 (Jagunal-homolog1) is a ER-resident transmembrane protein which is part of the early secretory pathway and granulocyte colony-stimulating factor receptor mediated signaling. Autosomal recessively inherited variants in the JAGN1 gene lead to congenital neutropenia, early-onset bacterial infections, aphthosis and skin abscesses due to aberrant differentiation and maturation of neutrophils. In addition, bone metabolism disorders and a syndromic phenotype, including facial features, short stature and neurodevelopmental delay, have been reported in affected patients.
View Article and Find Full Text PDFCells
December 2024
Departments of Blood and Marrow Transplant, Royal Manchester Children's Hospital, Manchester M13 9WL, UK.
Myeloid chimerism better reflects donor stem cell engraftment than whole-blood chimerism in assessing graft function following allogeneic hematopoietic stem cell transplant (HCT). We describe our experience with 130 patients aged younger than 18 years, treated with allogeneic HCT using bone marrow or PBSC from HLA-matched donors for non-malignant diseases, whose pre-transplant conditioning therapy included alemtuzumab and who were monitored with lineage-specific chimerism after transplant. At 6 years post-transplant, overall survival (OS) was 91.
View Article and Find Full Text PDFCells
December 2024
Research Institute of Fundamental and Clinical Immunology, 14, Yadrintsevskaya st., 630099 Novosibirsk, Russia.
Hematopoietic stem cell transplantation (HSCT) is a standard method for treating a number of pathologies, primarily blood diseases. Timely restoration of the immune system after HSCT is a critical factor associated with the development of complications such as relapses or secondary tumors and various infections, as well as the graft-versus-host reaction in allogeneic transplantation, which ultimately affects the survival of patients. Introduction into the recipient's body of immune system cells that are incapable of sensitization by recipient antigens during the period of immune reconstitution can increase the rate of restoration of the immune system, as well as reduce the risk of complications.
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