Commonconditions associated with deficiency in the complement - TopicsExpress

Commonconditions associated with deficiency in the complement components include increased susceptibility to bacterial infections and systemic lupus erythematosus which is related to the inability to clear immune complexes. Deficiency in the proteins that regulate complement activity can cause equally serious disorders. An example is paroxymal nocturnal hemoglobinuria, or PNH, which manifests as increased fragility of erythrocytes, leading to chronic hemolytic anemia, pancytopenia (loss of blood cells of all types) and venous thrombosis (formation of blood clots). The name PNH derives from the presence of hemoglobin in the urine, most commonly observed in the first urine passed after a night’s sleep. The cause of PNH is a general defect in synthesis of cell-surface proteins, which affects the expression of two regulators of complement, DAF (decay accelerating factor or CD55) and MIRL (membrane inhibitor of reactive lysis or CD59

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