Defects in cell-mediated immunity Many different stages of T-Cell development can result from abnormalities in what stages? What types of defects can result in Molecular defects severe combined immunodeficiency (SCID)? Besides primary defects in Can also have secondary effects on humoral immunity what other immunity. effects can happen? In general, defects in cellular Defects in humoral immunity immunity are more difficult to manage than what other defects? Patients with severe defects in They can develop graft-versus-host (GvH) disease. immunity may develop what disease? This happens when the donor A severe defect in the T-cell system. lymphocytes are allowed to survive, proliferate, and attack the recipient’s tissue as foreign. When can GvH disease occur? GvH disease can in any individual with a severe defect in cell-mediated immunity (e.g., in bone marrow transplant recipients) and can be fatal. GvH disease also occurs in patients who A bone marrow transplant. have received this type of transplant. This anomaly affects thymic DiGeorge anomaly is a developmental abnormality development. of the third and fourth pharyngeal pouches that affects thymic development. All organs derived from these structures Embryonic structures can be affected. DiGeorge anomaly associated Mental retardation, absence of ossification of the abnormalities include what? hyoid bone, cardiac anomalies, abnormal facial development, and thymic hypoplasia. The severity and extent of the DiGeorge anomaly developmental defect can be quite variable in this disease. The immunodeficiency associated Thymocytes DiGeorge anomaly is a quantitative defect in what type of cells? Insufficient types of these are made, but Mature T cells those that are present are functionally normal. One immunodeficiency for which a Purine nucleoside phosphorylase (PNP) deficiency, a specific enzymatic basis has been rare autosomal recessive trait. defined is what? The condition presents in what age group The condition presents in infancy and has recurrent or chronic pulmonary
Defects in cell-mediated immunity can result from abnormalities in what stages? Many different stages of T-Cell development What types of defects can result in severe combined immunodeficiency (SCID)? Molecular defects Besides primary defects in immunity what other effects can happen? Can also have secondary effects on humoral immunity. In general, defects in cellular immunity are more difficult to manage than what other defects? Defects in humoral immunity Patients with severe defects in immunity may develop what disease? They can develop graft-versus-host (GvH) disease. This happens when the donor lymphocytes are allowed to survive, proliferate, and attack the recipient’s tissue as foreign. A severe defect in the T-cell system. When can GvH disease occur? GvH disease can in any individual with a severe defect in cell-mediated immunity (e.g., in bone marrow transplant recipients) and can be fatal. GvH disease also occurs in patients who have received this type of transplant. A bone marrow transplant. This anomaly affects thymic development. DiGeorge anomaly is a developmental abnormality of the third and fourth pharyngeal pouches that affects thymic development. All organs derived from these structures can be affected. Embryonic structures DiGeorge anomaly associated abnormalities include what? Mental retardation, absence of ossification of the hyoid bone, cardiac anomalies, abnormal facial development, and thymic hypoplasia. The severity and extent of the developmental defect can be quite variable in this disease. DiGeorge anomaly The immunodeficiency associated DiGeorge anomaly is a quantitative defect in what type of cells? Thymocytes Insufficient types of these are made, but those that are present are functionally normal. Mature T cells One immunodeficiency for which a specific enzymatic basis has been defined is what? Purine nucleoside phosphorylase (PNP) deficiency, a rare autosomal recessive trait. The condition presents in what age group and has recurrent or chronic pulmonary The condition presents in infancy
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