C1-esterase inhibitor concentrate rescues erythrocytes from complement-mediated destruction in autoimmune hemolytic anemia

C1-esterase inhibitor concentrate rescues erythrocytes from complement-mediated destruction in autoimmune hemolytic anemia.

In autoimmune hemolytic anemia (AIHA), autoantibody-mediated complement activation results in C3 deposition on RBCs and possibly the formation of the membrane attack complex cumulating in intravascular hemolysis.1-4 In case of acute signs of tissue hypoxia, life-saving transfusion with RBC units is required. However, recovery of RBC transfusions is inadequate because autoantibodies react with r...

Role of Complement in Autoimmune Hemolytic Anemia.

The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglu...

Complement in hemolytic anemia.

Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributa...

Autoimmune Hemolytic Anemia

Reticulocytopenia in autoimmune hemolytic anemia.

ETICULOCYTOSIS is gemserally regarded as a characteristic feature of the hensolytic symsdrome. The outpouring of young red cells is ouse of the bone marrow’s reactiomss to the challenge of aisemia, atsd the numssber of reticulocytes it produces is oftets a good measure of the severity of the hemolytic process. Whets the red cell life spams is very short mssost of the cells ims the circulation n...