第二十一单元 免疫缺陷性疾病（Twenty-first unit immunodeficiency disease）.docVIP

第二十一单元 免疫缺陷性疾病（Twenty-first unit immunodeficiency disease） Twenty-first unit immunodeficiency disease Concept: immunodeficiency disease (IDD) is the innate immune system hypoplasia or acquired damage to immune cell development, proliferation and differentiation, metabolic abnormalities, immune dysfunction and lead to the emergence of the clinical syndrome. According to etiology, primary immunodeficiency disease (PIDD) and acquired immunodeficiency disease (SIDD) can be divided into two categories. Features: easy recurrence and persistent infection. Malignant tumor is easy to happen. Concomitant autoimmune disease. There is genetic predisposition. I. concept Primary immunodeficiency disease is a disease caused by immune system abnormalities, genetic abnormalities, or congenital immune system disorders resulting from immune dysfunction. Two, common diseases Classification diseases, immunodeficiency, pathogenesis, clinical features B cell Deficiency disease X linked non gamma globulin (XLA) Immature B cell BtK defect Extracellular bacteria, viral infections Selective IgA defect Low or no IgA Undetermined respiratory tract infection Sex linked high IgM syndrome (XHM) No Ig class conversion CD40L defect Infection by extracellular bacteria T cell Deficiency DiGeorge syndrome T and B cell development Thymic and multiple organ development disorder Recurrent intracellular parasite infection TCR signaling disorder T cell response deficit CD3 epsilon or gamma chain defect Infection by intracellular parasites Combined type exemption Epidemic disease Severe combined immune deficiency disease (SCID) 1.X- linked recessive inheritance SCID No T cell IL-2R gamma (gamma C) strand deletion Prone to repeated infection 2 autosomal recessive inheritance SCID Adenylate deaminase (ADA) and purine nucleotide phosphorylase (PNP) deficiency Disorders of T and B cell metabolism ADA and PNP defects Prone to repeated serious infection II. HLA class II molecular expression defect (naked lymphocyt