hematology and oncology
Autoimmune Hemolytic Anemia
Treatment of Autoimmune Hemolytic Anemia
Blood transfusion for severe, life-threatening anemia (usually with reticulocytopenia). For drug-induced warm antibody hemolytic anemia, drug withdrawal and sometimes IV immune globulin For idiopathic warm antibody hemolytic anemia, corticosteroids and, in refractory cases,rituximab, IV immune globulin, or splenectomy For cold agglutinin disease, avoidance of cold and treatment of underlying disorder For PCH, avoidance of cold, immunosuppressants, and treatment of syphilis if present. In children, this disease is often self-resolving. Blood transfusion is the most important treatment for symptomatic patients who rapidly develop severe, life-threatening anemia. In this situation, transfusion should never be withheld due to lack of "compatible" units. In general, patients who have not had a previous blood transfusion or been pregnant are at low risk for hemolysis of ABO-compatible blood. Even if transfused cells are hemolyzed, their hemolysis will be slower than the patient's own cells, so blood transfusion can be life-saving until more definitive therapy can be done. More specific treatment depends on the mechanism of the hemolysis. Indrug-induced warm antibody hemolytic anemias,drug withdrawal decreases the rate of hemolysis. Withmethyldopa-type AIHA, hemolysis usually ceases within 3 weeks; however, a positive antiglobulin test may persist for>1 year. With hapten-mediated AIHA, hemolysis ceases when the drug is cleared from the plasma. Corticosteroids and/or infusions of immune globulin may be used as second-line therapies. Inidiopathic warm antibody AIHA,corticosteroids (eg,prednisone1 mg/kg orally once a day) are the standard first-line treatment. When stable RBC values are achieved, the corticosteroids are tapered slowly with laboratory monitoring of hemolysis (eg, by hemoglobin and reticulocyte counts). The goal is to wean the patient completely from corticosteroids or to maintain remission with the lowest possible corticosteroid dose. About two thirds of patients respond to corticosteroid treatment. In patients who relapse after corticosteroid cessation or who are refractory to corticosteroids,rituximabis usually used as a second-line drug. Other treatments include use of additional immunosuppressive drugs,folic acid, and/or splenectomy. About one third to one half of patients have a sustained response after splenectomy. In cases of fulminant hemolysis, immunosuppression with high-dose pulse corticosteroids orcyclophosphamidecan be used. For less severe but uncontrolled hemolysis, immune globulin infusions have provided temporary control. Long-term management with immunosuppressants (includingcyclosporine) has been effective in patients in whom corticosteroids and splenectomy have been ineffective. The presence of panagglutinating antibodies in warm antibody hemolytic anemia makes cross-matching of donor blood difficult. In addition, transfusions could superimpose an alloantibody on the autoantibody, accelerating hemolysis. Thus, transfusions should be avoided when anemia is not life-threatening but should not be withheld in patients with severe autoimmune hemolytic anemia, particularly when the reticulocyte count is low. In many cases, avoidance of cold environments and other triggers of hemolysis may be all that is needed to prevent symptomatic anemia. , an inhibitor of the classical complement pathway, was shown to increase hemoglobin levels and decrease transfusion requirements in about half of patients with cold agglutinin disease ( Treatment reference ). It is a treatment option in patients with severe anemia. In severe cases, Plasmapheresis is an effective temporary treatment. Transfusions should be given sparingly, with the blood warmed through an on-line warmer. Splenectomy is usually of no value, and immunosuppressants have only modest effectiveness. In paroxysmal cold hemoglobinuria (PCH), therapy consists of strict avoidance of exposure to cold. Immunosuppressants have been effective, but use should be restricted to patients with progressive or idiopathic cases. Splenectomy is of no value. Treatment of concomitant syphilis may cure PCH. 1.Roth A, Barcellini W, D'Sa S, et al:Sutimlimabin cold agglutinin disease.N Engl J Med384(14):1323–1334, 2021. doi: 10.1056/NEJMoa2027760 