Severe combined immunodeficiency: case report of alogenic, haploidentical bone marrow transplantation
نویسندگان
چکیده
Methods Case report of a 2 years old male patient with severe combined immunodeficiency (SCID), diagnosed at 9 months after hospitalization due to failure to thrive, chronic diarrhea and pneumonia. Evolved with recurrent respiratory and gastrointestinal infections although using prophylaxis and immunoglobulin infusion. Alogenic, haploidentical transplantation was carried out with positive selection of CD34+, at 18 months of age due no compatible donor been found.
منابع مشابه
HLA-haploidentical bone marrow transplantation for severe combined immunodeficiency using E rosette fractionation and cyclosporine.
Eight patients with severe combined immunodeficiency received bone marrow cells from their HLA haplotype-identical fathers after bone marrow T cell depletion by rosetting with neuraminidase-treated sheep red cells. Because the method led to the infusion of a small percentage of T lymphocytes (0.1% to 0.3%), cyclosporin was given by continuous intravenous infusion for two months in order to prev...
متن کاملDevelopment of gene therapy: potential in severe combined immunodeficiency due to adenosine deaminase deficiency
A 4-month-old male received a T-lymphocyte-depleted haploidentical bone marrow transplant (BMT) for correction of severe combined immunodeficiency (SCID) due to adenosine deaminase (ADA) deficiency. Although previous haploidentical bone marrow transplants have been attempted in ADA-deficient SCID, complete reconstitution of both B-lymphocyte and T-lymphocyte function has not been obtained after...
متن کاملBone marrow transplantation in leukemia in the absence of an HLA-identical sibling donor.
Bone marrow transplantation is a potentially curative treatment for patients with leukemia, aplastic anemia, metabolic disorders, and immunodeficiency. One of the main problems ofthis procedure is the graftversus-host reaction induced in the recipient by the immune function of the transplanted marrow. In order to minimize the risks of graft rejection and graft-versus-host disease (GVHD), marrow...
متن کاملAtypical Omenn Syndrome Due to RAG2 Gene Mutation, a Case Report
Severe Combined Immunodeficiency (SCID), characterized by a profound decrease in both the number and function of T cells, is related to more than 20 different mutations. Recombination-activating gene (RAG) 1 and 2 seem to be two of the most common forms presenting with various manifestations, including typical SCID, Omenn syndrome (OS), atypical SCID (AS), or delayed onset combined immunodefici...
متن کاملBone Marrow Transplantation in Major Histocompatibility Complex Class I1 Deficiency: A Single-Center Study
Major histocompatibility complex (MHC) class II deficiency (bare lymphocyte syndrome) is a rare inborn error of the immune system characterized by impaired antigen presentation and combined immunodeficiency. It causes severe and unremitting infections leading to progressive liver and lung dysfunctions and death during childhood. As in other combined immunodeficiency disorders, bone marrow trans...
متن کامل