Haemophagocytic lymphohistiocytosis in X-linked severe combined immunodeficiency
نویسندگان
چکیده
منابع مشابه
Safety Modality for X-linked Severe Combined Immunodeficiency Gene Therapy
Abbreviations: SCID-X1: X-linked Severe Combined Immunodeficiency; γc: Cytokine Receptor Common Gamma Chain; HSC: Hematopoietic Stem Cells; ab: Antibody; wt: Wild-type; NK cell: Natural Killer Cell; HLA: Human Leukocyte Antigen; SIN: Selfinactivating; HSV-TK: Thymidine kinase of Herpes Simplex Virus; ER: Endoplasmatic Reticulum; MFI: Mean Fluorescence Intensity; CDC: Complement-dependent Cytoto...
متن کاملEfficacy of gene therapy for X-linked severe combined immunodeficiency.
BACKGROUND The outcomes of gene therapy to correct congenital immunodeficiencies are unknown. We reviewed long-term outcomes after gene therapy in nine patients with X-linked severe combined immunodeficiency (SCID-X1), which is characterized by the absence of the cytokine receptor common gamma chain. METHODS The nine patients, who lacked an HLA-identical donor, underwent ex vivo retrovirus-me...
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Canine X-linked severe combined immunodeficiency (XSCID) is due to mutations in the common gamma chain which is a subunit of the receptors of IL-2, IL-4, IL-7, IL-9 and IL-15. Bone marrow transplantation (BMT) of human XSCID patients without pretransplant conditioning (cytoablation) results in engraftment of donor T-cells and reconstitution of T-cell function but engraftment of few, if any, don...
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X-linked severe combined immunodeficiency (XSCID) is a lethal disease caused by a defect in the gene encoding the common gamma chain (gamma-c) of the receptor for interleukin-2 (IL-2), IL-4, IL-7, IL-9, and IL-15. Allogeneic bone marrow transplantation, the current therapy of choice for this defect, is often complicated by graft-versus-host disease and/or incomplete reconstitution of B-lymphocy...
متن کاملReview of haemophagocytic lymphohistiocytosis.
Haemophagocytic lymphohistiocytosis (HLH) describes a clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response. It may develop subsequent to a number of recognised genetic mutations or in association with infection, malignancy, autoinflammatory or metabolic conditions. Even with the published diagnostic criteria it can be difficult to make the diagnosi...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 2000
ISSN: 0007-1048
DOI: 10.1046/j.1365-2141.2000.01923.x