Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review.

摘要: Autoimmune haemolytic anaemia (AIHA) is an immune disorder caused by antibodies directed against unmodified autologous red cells. The may be a primary (idiopathic) or secondary disease. diagnosis based on the presence of anaemia, signs haemolysis with reticulocytosis, low haptoglobin, increased lactate dehydrogenase, elevated indirect bilirubin, and positive direct antiglobulin test (Coombs test). Sometimes, not all these typical features are present. Most AIHA warm antibodies, whereas cold less commonly detected. While half antibody-based idiopathic anaemias, almost antibody anaemias. Underlying diseases Non Hodgkin's lymphomas systemic autoimmune disorders, frequently organ transplantation, infections, solid tumors. Moreover, important complication treatment nucleoside analogs. patients require therapy. In AIHA, standard first line therapy glucocorticosteroids without high dose immunoglobulins, splenectomy considered second-line Response rates to corticosteroid high. After initial remission, should tapered down slowly caution, in some cases, low-dose maintenance required. efficacy that develops lymphoma patients, posttransplant tumor patients. Among other immunosuppressive treatments, rituximab (anti-CD20) appears highly effective refractory Mycophenolate mofetil quite underlying lymphoproliferative Patients agglutinins steroids splenectomy. Half respond rituximab, although responses usually short-lived. associated malignant tumors, disappears after successful anti-lymphoma anti-tumor