Autoimmune Haemolytic Anaemia Treated with Multiple Transfusions, Immunosuppressive Therapy, Plasma Exchange, and Desferrioxamine

Abstract

Severe autoimmune haemolytic anaemia in a five year old boy was unaffected by treatment with prednisone and splenectomy, but subsided after combined immunosuppressive therapy and three plasma exchanges. Over five months, a total of 93 transfusions of concentrated erythrocytes was given (equal to 18.6 grams of iron or 1.1 g/kg BW). This resulted in severe iron overload with cardiac, hepatic, and pancreatic complications, together with growth-retardation. These complications disappeared after treatment with desferrioxamine and vitamin C, but despite a normal growth hormone response to glucagon the concentration of somatomedin in serum remained low. Treatment by plasma exchanges and immunosuppressive agents may therefore be of value in severe haemolytic anaemia refractory to corticosteroids and splenectomy. Iron chelating therapy should be considered if multiple transfusions result in iron overload.

Intensive plasma exchange for severe autoimmune hemolytic anemia in a four-month-old infant

“…Plasma exchange (PE) removes autoantibodies from patients with severe autoimmune hemolytic anemia (AIHA) and allows time for more conventional therapies to become effective [8,2,1]. AIHA has been associated with significant mortality in children; 10% to 28% [4,7,23,3].…”

Plasma Exchange Treatment in Autoimmune Hemolytic Anemia of the Warm Antibody Type with Renal Failure

“…The role of plasmapheresis in the treatment of several immune-complex disorders is well accepted, but in the field of antibody-mediated hematologic disorders, plas ma exchange (PE) has been used with controversial results [1,2], In patients with autoimmune hemolytic anemia (AIHA) of the cold antibody type PE has been employed in occasional cases with little efficiency [3][4][5][6], on the other hand, it had some benefit in AIFIA of the warm antibody type [7][8][9][10][11]. We here report on an additional case with beneficial effect of PE in a black patient with sickle cell trait, who developed an extremely severe form of AIHA of the warm antibody type with acute renal failure, refractory to conventional treatment with corti costeroids.…”

Therapeutic Plasma Exchange

“…Cold agglutinins are usually IgM antibodies directed against the I/i antigens; they bind preferentially at low temperatures and produce the syndrome of complement-mediated intravascular hemolysis known as cold agglutinin disease (CAD). [282][283][284][285][286][287][288] IgM antibodies in CAD are predominantly intravascular and only loosely bound to cells; thus their removal by TPE should be relatively efficient. They bind better at body temperature and produce a predominantly extravascular hemolytic syndrome (warm autoimmune hemolytic anemia [WAHA]).…”