Hematopoietic stem cell transplantation for the treatment of leukocyte adhesion deficiency type III
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چکیده
منابع مشابه
Hematopoietic Stem Cell Transplantation for Thalassemia
Thalassemia is an autosomal recessive disorder associated with defective synthesis of the α- or β-chain of hemoglobin. For β-thalassemia major patients, therapeutic options are either monthly red cell transfusions and chelation therapy or allogeneic stem cell transplant. Stem cell transplant is the only curative approach and success is inversely correlated with the degree of iron overload and h...
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The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
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The genetic disease canine leukocyte adhesion deficiency (CLAD) is characterized by recurrent, severe bacterial infections, typically culminating in death by 6 months of age. CLAD is due to a mutation in the leukocyte integrin CD18 subunit, which prevents surface expression of the CD11/CD18 leukocyte integrin complex. We demonstrate that stable mixed donor:host hematopoietic chimerism, achieved...
متن کاملhematopoietic stem cell transplantation for thalassemia
thalassemia is an autosomal recessive disorder associated with defective synthesis of the α- or β-chain of hemoglobin. for β-thalassemia major patients, therapeutic options are either monthly red cell transfusions and chelation therapy or allogeneic stem cell transplant. stem cell transplant is the only curative approach and success is inversely correlated with the degree of iron overload and h...
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ژورنال
عنوان ژورنال: Pediatrics & Neonatology
سال: 2017
ISSN: 1875-9572
DOI: 10.1016/j.pedneo.2017.07.006