Autoimmune Hemolytic Anemias

Silberstein, Leslie E.; Cunningham, Melody J.

doi:10.1016/b978-0-443-06981-9.50046-6

Cited by 10 publications

(4 citation statements)

References 150 publications

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“…3 AIHA is very rare in infancy and childhood (0.2 per 10 5 /year), 4 where it is primary in 37% and associated with immune disorders in 53% of cases. Mortality is lower in children (4%), but rises to 10% if the hemolytic anemia is associated with immune thrombocytopenia (Evans syndrome).…”

Section: Introductionmentioning

confidence: 99%

Treatment of autoimmune hemolytic anemias

2014

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Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. The first-line therapy for warm AIHA are corticosteroids, which are effective in 70-85% of patients and should be slowly tapered over a time period of 6-12 months. For refractory/relapsed cases, the current sequence of second-line therapy is splenectomy (effective approx. in 2 out of 3 cases but with a presumed cure rate of up to 20%), rituximab (effective in approx. 80-90% of cases), and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases. In CAD, rituximab is now recommended as first-line treatment. ABSTRACT Treatment of warm AIHAThe traditional treatment of AIHA includes corticosteroids, splenectomy and conventional immunosuppressive drugs. Over recent years, some new therapies have become available and there has been some evidence of success. These therapies are primarily used in patients who are not candidates for or fail to respond to splenectomy, those who relapse after splenectomy, and those who cannot maintain stable hemoglobin levels without unacceptably high doses of corticosteroids. First-line therapy CorticosteroidsThere is general agreement that corticosteroids represent the first-line treatment for patients with warm antibody type AIHA, albeit their use is based on experience rather than hard evidence. In fact, there is little published information on their effectiveness, 1,16,17 and this is not supported by clinical trials. Corticosteroids, usually prednisone, are given at the initial dose of 1.0-1.5 mg/kg/day for 1-3 weeks until hemoglobin levels greater than 10 g/dL are reached. Response occurs mainly during the second week, and if none or minimal improvement is observed in the third week, this therapy is assumed to be ineffective. After stabilization of hemoglobin, prednisone should be gradually and slowly tapered off at 10-15 mg weekly to a daily dose of 20-30 mg, then by 5 mg every 1-2 weeks until a dose of 15 mg, and subsequently by 2.5 mg every two weeks with the aim of withdrawing the drug. Although one might be tempted to discontinue steroids more rapidly, AIHA patients should be treated for a minimum of three or four months with low doses of prednisone (≤10 mg/day).1 In fact, patients receiving low doses of cortico...

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Section: Introductionmentioning

confidence: 99%

Treatment of autoimmune hemolytic anemias

2014

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“…It is worth mentioning that the strength of DAT positivity doesn’t correlate with the severity of hemolysis. Yet, DAT specificity together with the antibody thermal amplitude of reactivity helps to further subclassify the AIHA into warm (IgG only, IgG and C3 or C3 only in 20%, 67% and 13% of cases, respectively), cold (C3 with high titer, and high thermal amplitude cold agglutinins) mixed (IgG, C3 with high thermal amplitude cold agglutinins) and PCH (C3 along with positive specific Donath Landsteiner test) [ 4 , 14 , 15 ]. In addition, obtaining the acid eluate by detaching antibodies from RBCs should be performed to detect autoantibody specificity, although most autoantibodies typically exhibit panreactivity.…”

Section: Discussionmentioning

confidence: 99%

Rare case of refractory mixed autoimmune hemolytic anemia in a 6-year-old child: a case report

Hanna,

Carcao

2023

J Med Case Reports

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Background Mixed autoimmune hemolytic anemia (AIHA) shows combined clinical and laboratory characteristics of warm and cold AIHA. It is relatively uncommon in children. Consequently, knowledge about mixed AIHA prevalence, clinical presentation, treatment options, and prognosis in children is limited to very few case reports. Case presentation We describe a six-year-old Asian girl presenting with profound anemia, blood group typing discrepancy and crossmatch incompatibility, post upper respiratory tract infection. Detection of red cell warm and cold reactive autoantibodies, led to the diagnosis of mixed AIHA. Autoantibodies with laboratory evidence of hemolysis persisted despite high dose steroid therapy. Due to the inability to wean further, the patient was subsequently commenced on mycophenolate mofetil to which she seems to be responding. Conclusions Mixed AIHA may be notoriously difficult to diagnose and treat. Detailed clinical and laboratory work-up is essential to establish the diagnosis. To the best of our knowledge, this is the first case report of mixed AIHA following upper respiratory tract infection. Awareness of this occurrence is important, as similar to warm AIHA, mixed AIHA should be treated immediately by early initiation of steroid therapy. In addition, prompt supportive care as well as long-term clinical follow-up are required to improve outcomes of these cases.

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“…Highly related codes were presented adjacent to each other in Tables 3 through 10 and subtotaled. All 524 diagnoses in the tables met one of the following criteria 32–85 :…”

Section: Methodsmentioning

confidence: 99%

Use of Diagnosis Codes to Find Blood Transfusion Adverse Events in Electronic Health Records

et al. 2022

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Objective: Electronic health records (EHRs) and big data tools offer the opportunity for surveillance of adverse events (patient harm associated with medical care). We used International Classification of Diseases, Ninth Revision, codes in electronic records to identify known, and potentially novel, adverse reactions to blood transfusion. Methods:We used 49,331 adult admissions involving critical care at a major teaching hospital, 2001-2012, in the Medical Information Mart for Intensive Care III EHRs database. We formed a T (defined as packed red blood cells, platelets, or plasma) group of 21,443 admissions versus 25,468 comparison (C) admissions. The International Classification of Diseases, Ninth Revision, Clinical Modification, diagnosis codes were compared for T versus C, described, and tested with statistical tools.Results: Transfusion adverse events (TAEs) such as transfusion-associated circulatory overload (TACO; 12 T cases; rate ratio [RR], 15.61; 95% confidence interval [CI], 2.49-98) were found. There were also potential TAEs similar to TAEs, such as fluid overload disorder (361 T admissions; RR, 2.24; 95% CI, 1.88-2.65), similar to TACO. Some diagnoses could have been sequelae of TAEs, including nontraumatic compartment syndrome of abdomen (52 T cases; RR, 6.76; 95% CI, 3.40-14.9) possibly being a consequence of TACO.Conclusions: Surveillance for diagnosis codes that could be TAE sequelae or unrecognized TAE might be useful supplements to existing medical product adverse event programs.

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Autoimmune Hemolytic Anemias

Cited by 10 publications

References 150 publications

Treatment of autoimmune hemolytic anemias

Treatment of autoimmune hemolytic anemias

Rare case of refractory mixed autoimmune hemolytic anemia in a 6-year-old child: a case report

Use of Diagnosis Codes to Find Blood Transfusion Adverse Events in Electronic Health Records

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