ANEMIA HEMOLITICA AUTOINMUNE PDF

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A rare, autoimmune disorder in which various types of auto-antibodies are directed against red blood cells causing their survival to be shortened and resulting in hemolytic anemia.

AIHA can be primary idiopathic , secondary to infection or associated with diseases such as B-cell lymphomas, other systemic or organ-specific autoimmune diseases, Hodgkin's disease, hepatitis or primary immunodeficiencies, or, in the case of drug-induced AIHA, caused by a reaction to drugs. The condition may develop gradually or occur suddenly. Diagnosis is based on clinical or laboratory evidence of hemolysis and the detection of autoantibodies by means of the direct anti-globulin test DAT.

Treatment is dependent on correct diagnosis. Cases of drug-induced AIHA should be investigated to determine if stopping a drug will induce remission. For warm AIHAs, corticosteroids are used, followed by splenectomy if necessary. Some targeted therapies, such as rituximab anti-CD20 monoclonal antibody , have shown promising results. Other immunosuppressive treatments may be suggested.

For cold AIHA, keeping the patient warm may be sufficient, and corticosteroids and splenectomy must be avoided as they are known to be ineffective. Rituximab has been demonstrated as a relatively effective and safe option for treating patients with symptomatic chronic cold agglutinin disease.

Transfusion may be necessary in cases with inadequate response to therapy and life-threatening worsening anemia. However, transfusion can be complicated because of the presence of the autoantibodies, which can, in addition, increase destruction of the donor red blood cells. Prognosis depends on the underlying cause of the disease and whether symptoms are managed appropriately and in a timely manner, but death is a rare outcome.

Other search option s Alphabetical list. Suggest an update. Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition A rare, autoimmune disorder in which various types of auto-antibodies are directed against red blood cells causing their survival to be shortened and resulting in hemolytic anemia.

Etiology AIHA can be primary idiopathic , secondary to infection or associated with diseases such as B-cell lymphomas, other systemic or organ-specific autoimmune diseases, Hodgkin's disease, hepatitis or primary immunodeficiencies, or, in the case of drug-induced AIHA, caused by a reaction to drugs.

Diagnostic methods Diagnosis is based on clinical or laboratory evidence of hemolysis and the detection of autoantibodies by means of the direct anti-globulin test DAT. Differential diagnosis Biological differential diagnoses include other non-autoimmune causes of hemolytic anemia. Management and treatment Treatment is dependent on correct diagnosis.

Prognosis Prognosis depends on the underlying cause of the disease and whether symptoms are managed appropriately and in a timely manner, but death is a rare outcome. Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 0. Health care resources for this disease Expert centres Diagnostic tests 17 Patient organisations 16 Orphan designation s and orphan drug s 7.

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