Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person’s own red blood cells (RBCs) cause them to burst (lyse), leading to. Autoimmune hemolytic anemia (AIHA) is an uncommon disorder characterized by hemolysis mediated by autoantibodies directed against. Autoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either.
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Diagnosis and classification of autoimmune hemolytic anemia.
However, in those cases mediated by drug-induced autoantibodies, the DAT may remain positive for weeks to months after discontinuation of the offending drug and cessation of hemolysis. Autoanti-Ge associated with severe autoimmune hemolytic anemia. Rather, AIHA should be considered secondary only when i AIHA and the suspect disease occur together more frequently than by chance alone, ii AIHA remits with correction of the suspect disease, autoimhn iii the suspect disease causes immunologic aberration [ 3 ].
Proc R Soc Med. During the past three decades, studies defining red-cell blood groups and serum antibodies have produced diagnostic methods that have laid the basis for immunologic concepts relevant to many of the acquired hemolytic states. Idiopathic primary chronic cold agglutinin disease associated with clonal B-lymphocyte proliferation with or without symptomatic lymphoma. Unusual clinical presentations of AIHA may cause initial confusion in the diagnosis.
Other patients with established lymphoproliferative disorders may develop cold agglutinin disease as a manifestation of hemolitikk lymphoma. Data mengenai karakteristik demografi dan respon pengobatan AHAI, khususnya pemberian kortikosteroid belum banyak didapatkan di Indonesia.
Autoimmune hemolytic anemia: From lab to bedside
Less common causes of warm-type AIHA include neoplasms other than lymphoid, and infection. On the other hand, IgM antibodies readily activate the classical complement pathway and produce cytolysis. This article has been cited by other autoiun in PMC. Hematology of Infancy and Childhood. Hypersensitivity and autoimmune diseases It is more common in women than in men [ 23 ].
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 ]. Serological characterization of autoantibody helps to differentiate various types of AIHA and gives a better assessment to the clinician regarding the likely course of disease adalwh the form of treatment to be given.
In very severe cases, including those with acute onset, patients may present with fever, pallor, jaundice, fig. These reactions may result from unrelated antigen-antibody complexes that fix to an innocent-bystander erythrocyte, or from related antigen-antibody combinations in which the host red cell or some part of its structure is or has become antigenic.
Published online Sep To determine the autoantibody or autoantibodies present in a patient, the Coombs testalso known as the antiglobulin test, is performed.
The Clinical Pictures of Autoimmune Hemolytic Anemia
Anti-i specificity is predominant in patients with infectious mononucleosis and in some patients with lymphoma. Asian Pac J Allergy Immunol. Drug-induced immune hemolytic anemia is usually mild, and the prognosis is good. In addition pathologic cold autoantobodies generally have a titer of greater than 1: Sometimes there is evidence of an underlying lymphoproliferative disorder.
The majority of these patients exhibit clonal B-lymphocyte proliferation [ 8 ]. Chaudhary and Sudipta Sekhar Das 1.
No critical patient should be denied blood transfusion due to serological incompatibility. AIHA is classified hemolitio either warm autoimmune hemolytic anemia or cold autoimmune hemolytic anemiawhich includes cold agglutinin disease and paroxysmal cold hemoglobinuria. Congenital or tertiary syphilis in adults. Primary warm antibody AIHA accounts for about half of the cases [ 34 ].
Autoimmune hemolytic anemia
Sang Hyuk ParkM. A broad-spectrum antiglobulin reagent detects both immunoglobulin and complement components on patient RBCs.
adalan Moreschlit had used the same method in in a goat autpimun system. Evans syndrome in childhood: In patients with refractory warm AIHA, administration of high dose cyclophosphamide, alemtuzumab anti-CD52 monoclonal antibodyor 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 [ 28 ].
Autoimmune hemolytic anemia and the Kell blood groups. The clinical picture is quite variable.
Recently, new therapeutic approaches such as the administration of rituximab hemilitik monoclonal antibodyerythropoiesis-stimulating agents, other immunosuppressive agents such as cyclosporine A CsAmycophenolate mofetil MMFdanazol synthetic anabolic steroid have become available and there has been increasing evidence of success.
The opposite is true of i antigens. Serology of acquired hemolytic anemia. However, transfusion should not be withheld in a autoimuun ill patient even in the absence of compatible blood. Red blood cell transfusion in patients with autoantibodies: Occasionally immune thrombocytopenia is seen in patients with warm antibody AIHA, a condition termed Evans syndrome [ 17 ].