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      The Clinical Pictures of Autoimmune Hemolytic Anemia

      review-article
      Author(s): *
      Publication date (Electronic):
      Journal: Transfusion Medicine and Hemotherapy
      Publisher: S. Karger AG
      Keywords: Autoimmunity, Hemolysis, Agglutinin, Hemolysin, Direct antiglobulin test

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          Summary

          Autoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either warm reactive or cold reactive. The rate of hemolysis and the severity of the anemia may vary from mild to severe and life-threatening. Diagnosis is made in the laboratory by the findings of anemia, reticulocytosis, a positive Coombs test, and specific serologic tests. The prognosis is generally good but renal failure and death sometimes occur, especially in cases mediated by drugs.

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          Most cited references57

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          Autoimmune haemolysis: an 18-year study of 865 cases referred to a regional transfusion centre.

          R Sokol, S Hewitt, B K Stamps (1981)
          Clinical and serological records of 865 patients with confirmed autoimmune haemolysis (AIH)--a much larger series than any previously reported--were critically reviewed nd analysed. A proposed new classification for AIH based on serological findings differs from traditional classifications in that a new category of "mixed" AIH has been defined in which both "warm" and "cold" autoantibodies are present, and both are capable of causing haemolysis. Patients in this mixed group tend to have severe disease that may run a chronic intermittent course. The presentation of cold agglutinin disease is much more variable than has been seen in previous studies, haemolysis due to low titre autoantibodies being common. The AIH associated with pregnancy, usually considered as being of bad prognosis, is often mild and self limiting in the absence of other associated disorders.
          Article 0 comments Cited 53 times – based on 0 reviews     Review now
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            Direct antiglobulin ("Coombs") test-negative autoimmune hemolytic anemia: a review.

            George Segel, Marshall Lichtman (2014)
            We have reviewed the literature to identify and characterize reports of warm-antibody type, autoimmune hemolytic anemia in which the standard direct antiglobulin reaction was negative but a confirmatory test indicated that the red cells were opsonized with antibody. Three principal reasons account for the absence of a positive direct antiglobulin test in these cases: a) IgG sensitization below the threshold of detection by the commercial antiglobulin reagent, b) low affinity IgG, removed by preparatory washes not conducted at 4°C or at low ionic strength, and c) red cell sensitization by IgA alone, or rarely (monomeric) IgM alone, but not accompanied by complement fixation, and thus not detectable by a commercial antiglobulin reagent that contains anti-IgG and anti-C3. In cases in which the phenotype is compatible with warm-antibody type, autoimmune hemolytic anemia and the direct antiglobulin test is negative, an alternative method to detect low levels of IgG sensitization, use of 4°C, low ionic strength washes to prepare the cells for the direct antiglobulin test reaction to permit retention and identification of low affinity IgG antibodies, and, if the latter are uninformative, testing for sensitization with an anti-IgA, and, if necessary, an anti-IgM reagent identifies cases of warm-antibody type, immune hemolysis not verified by a commercial reagent.
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              Auto-immune haemolytic anaemia--a high-risk disorder for thromboembolism?

              A M Hendrick (2003)
              An audit of the effect of anticoagulant prophylaxis in acute exacerbations of severe autoimmune haemolysis was undertaken. All cases of this disorder presenting to one institution over a 16 year period were reviewed. There were 28 patients who had a total of thirty six exacerbations of haemolysis. Anticoagulant prophylaxis had been introduced from 1992 following three cases with fatal pulmonary emboli. Venous thromboembolism occurred in 5 of 15 exacerbations without prophylaxis but in only one of 21 in which prophylaxis was given. It is suggested that auto-immune haemolysis increases the risk of thromboembolism but further studies are required to quantify the risks and to define optimal prophylactic regimens.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Transfus Med Hemother
                Journal ID (iso-abbrev): Transfus Med Hemother
                Journal ID (publisher-id): TMH
                Title: Transfusion Medicine and Hemotherapy
                Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                ISSN (Print): 1660-3796
                ISSN (Electronic): 1660-3818
                Publication date (Print): September 2015
                Publication date (Electronic): 11 September 2015
                Publication date PMC-release: 11 September 2015
                Volume: 42
                Issue: 5
                Pages: 317-324
                Affiliations
                Department of Hematology, Levine Cancer Institute, University of North Carolina School of Medicine, Carolinas Healthcare System, Charlotte, NC, USA
                Author notes
                *Prof. Dr. Charles H. Packman, Department of Hematology, Levine Cancer Institute, University of North Carolina School of Medicine, Carolinas Healthcare System, 1021 Morehead Medical Drive, Suite 5300, Charlotte, NC 28204, USA, cpackman@ 123456carolinas.org
                Article
                Publisher ID: tmh-0042-0317
                DOI: 10.1159/000440656
                PMC ID: 4678314
                SO-VID: d6ba9447-e441-47c2-aff7-a87dc6b6ea8a
                Copyright © Copyright © 2015 by S. Karger GmbH, Freiburg
                License:

                This is an open access article distributed under the terms of Karger's Author's Choice™ licensing agreement, adapted from the Creative Commons Attribution Non-Commercial 2.5 license. This license allows authors to re-use their articles for educational and research purposes as long as the author and the journal are fully acknowledged.

                History
                Date received : 1 June 2015
                Date accepted : 12 August 2015
                Page count
                Figures: 3, Tables: 2, References: 62, Pages: 8
                Categories
                Subject: Review Article

                Keywords: autoimmunity,hemolysis,agglutinin,hemolysin,direct antiglobulin test
                Data availability:
                Keywords: autoimmunity, hemolysis, agglutinin, hemolysin, direct antiglobulin test

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