Therapeutic targets
- Compensation of anemia
- Cure (see below beta-thalassemia).
Therapy recommendations
- Therapy for hemolytic anemia depends on the underlying disorder in each case.
- In many cases, additional blood transfusions are needed.
- See also under “Further therapy“.
Therapy an acute hemolytic crisis in the context of a transfusion incident (mistransfusion in the AB0 system):
- Immediate stop of the transfusion
- Symptomatic therapy: volume substitution, administration of glucocorticoids, and urinary alkalinization with sodium bicarbonate
- Ultima Ratio: exchange transfusion.
Therapy of hereditary hemolytic anemia/red cell membrane defects:
- In addition to the recommended splenectomy (surgical removal of the spleen), prophylaxis with folic acid should also be performed in cases of severe hemolysis. → the daily requirement is then about 300-400 μg; In manifest folic acid deficiency should be added 5-15 mg/d.
- Before splenectomy, vaccination against pneumococcus and H. influenzae should be performed.
Therapy of autoimmune hemolytic anemia by heat antibodies:
- Glucocorticoids
- In severe hemolysis, therapy with prednisolone 1 mg/kg bw/d, after Hb normalization (Hb = blood pigment) tapering of the dose.
- In patients who do not respond, therapy with immunosuppressants (drugs that reduce the functions of the immune system) or the administration of gamma globulins (synonym: immunoglobulin G (IgG); antibodies (immunoglobulins) of class G, which act mainly against viruses and bacteria) may be indicated.
Therapy of thalassemia (disorders of hemoglobin formation).
- To date, cure by allogeneic stem cell therapy.
- Gene therapy for beta-thalassemia; the patient’s stem cells are taken and given a correct version of the gene using lentiviruses in the laboratory before infusion.In a phase II study, 3 of 9 patients were able to completely avoid blood transfusions after gene therapy; the others had a significant reduction in transfusions (by 73% in the entire group). Since patients’ therapies now range from 15 to 42 months, the chances of cure are good.
