Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body. Severe disease may cause jaundice or abdominal discomfort and fullness due to splenomegaly an enlarged spleen. Treatment is corticosteroids or other drugs that suppress the immune system and sometimes, splenectomy surgical removal of the spleen. See also Overview of Anemia.
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The presentation of clinical symptoms is typically insidious over several months but some patients show acute severe symptoms. Presenting complaints of AIHA depends on the severity of anemia itself, ranging from asymptomatic compensated reticulocytosis with mild hyperbilirubinemia to acute fulminant hemolysis leading to jaundice, hematosplenomegaly, tachycardia and angina. Clinical features are determined by the presence of underlying diseases and degree of hemolysis, which depends on the autoantibody type.
The traditional treatment of warm AIHA was the use of corticosteroid as first-line therapy and conventional immunosuppressive drugs such as azathioprine and cyclophosphamide, or surgical splenectomy as second-line therapy in patients with insufficient response to corticosteroid.
Recently, new therapeutic approaches such as the administration of rituximab anti-CD20 monoclonal antibody , erythropoiesis-stimulating agents, other immunosuppressive agents such as cyclosporine A CsA , mycophenolate mofetil MMF , danazol synthetic anabolic steroid have become available and there has been increasing evidence of success.
However, its superior efficacy over other second-line treatment options has not been confirmed, and both the lack of reliable predictors for outcomes and many surgical complications including the risk of overwhelming sepsis hinder wide application of splenectomy [ 2 ]. Other therapeutic options such as danazol, CsA, MMF and erythropoiesis-stimulating agents have been reported to possess additional benefit in patients with warm AIHA, but their clinical benefits should be further validated in the future clinical trials.
In patients with refractory warm AIHA, administration of high dose cyclophosphamide, alemtuzumab anti-CD52 monoclonal antibody , or ofatumumab anti-CD20 monoclonal antibody that targets different epitope compared to rituximab can be considered as the "last option" treatments, although the toxicity of alemtuzumab hinder the wide application of this drug [ 2 , 8 ]. However, based on the belief that increased oxygen-carrying capacity provided by the transfused RBCs may be enough to satisfy patient's oxygen need until other treatment options become effective, RBC transfusion still has its clinical benefit as a supportive treatment option especially in patients with symptomatic cardiovascular diseases in which sufficient oxygen supply is important.
A recent study demonstrated that regardless of autoantibody type, DAT specificity, DAT strength and corticosteroid therapy status, the transfusion of "the least incompatible" RBC to patients with autoantibodies yielded similar hemoglobin levels without increases of hemolysis risk compared with those with alloantibodies only and those with no antibodies who were transfused compatible RBC [ 9 ].
In this issue of Blood Research , Prabhu et al. This study reflects the heterogeneity in the disease characteristics of AIHA, which requires further validation in terms of clinical characteristics and treatment outcome to various drugs used in patients with AIHA. In conclusion, the improvement of drugs enabled more stratified approach for patients with first-line corticosteroid-refractory AIHA.
It is current concept that the second-line therapy would be splenectomy and rituximab, and thereafter any of immunosuppressive drugs. RBC transfusion would be an effective supporting treatment option as a bridge therapy. Novel drugs can be considered as the final option in AIHA patients who are refractory to all other drugs. However, the choice of second or further-line therapies would depend on the clinician's personal experiences and opinions.
National Center for Biotechnology Information , U. Journal List Blood Res v. Blood Res. Published online Jun Sang Hyuk Park , M. Find articles by Sang Hyuk Park. Author information Copyright and License information Disclaimer. This article has been cited by other articles in PMC. References 1. How I treat autoimmune hemolytic anemias in adults. Zanella A, Barcellini W. Treatment of autoimmune hemolytic anemias.
Diagnosis and classification of autoimmune hemolytic anemia. Autoimmun Rev. An automatable format for accurate immunohematology testing by flow cytometry. Development of flow cytometry for detection and quantitation of red cell bound immunoglobulin G in autoimmune hemolytic anemia with negative direct Coombs test. Asian Pac J Allergy Immunol. New insights into childhood autoimmune hemolytic anemia: a French national observational study of children. Autoimmune hemolytic anemia with reticulocytopenia.
A medical emergency. Salama A. Treatment options for primary autoimmune hemolytic anemia: a short comprehensive review. Transfus Med Hemother. Red blood cell transfusion in patients with autoantibodies: Is it effective and safe without increasing hemolysis risk? Ann Lab Med. Clinical characteristics and treatment outcomes of primary autoimmune hemolytic anemia: a single center study from South India.
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Autoimmune hemolytic anemia
Hemolysis is usually extravascular. The direct antiglobulin direct Coombs test establishes the diagnosis and may suggest the cause. Treatment depends on the cause and may include corticosteroids, splenectomy, IV immune globulin , immunosuppressants, avoidance of blood transfusions except in cases of life-threatening anemia , avoidance of triggers eg, cold , and withdrawal of drugs. See also Overview of Hemolytic Anemia. Autoimmune hemolytic anemia is caused by abnormalities extrinsic to the red blood cell RBC. Warm antibody hemolytic anemia is the most common form of autoimmune hemolytic anemia AIHA ; it is more common among women. Autoimmune hemolytic anemia may be classified as.
Autoimmune hemolytic anemia: From lab to bedside
Autoimmune hemolytic anemia AIHA occurs when antibodies directed against the person's own red blood cells RBCs cause them to burst lyse , leading to an insufficient number of oxygen-carrying red blood cells in the circulation. The lifetime of the RBCs is reduced from the normal — days to just a few days in serious cases. The antibodies are usually directed against high-incidence antigens , therefore they also commonly act on allogenic RBCs RBCs originating from outside the person themselves, e. The terminology used in this disease is somewhat ambiguous.
Autoimmune Hemolytic Anemia
Autoimmune hemolytic anemia AIHA is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test DAT still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients.
Diagnosis and treatment of autoimmune hemolytic anemia: classic approach and recent advances