Caractéristiques des anémies hémolytiques auto-immunes de l’adulte : analyse rétrospective d’une série de 83 patients

Genty, I.; Michel, Marc; Hermine, Olivier; Schaeffer, A; Godeau, Bertrand; Rochant, H

doi:10.1016/s0248-8663(02)00688-4

Cited by 76 publications

(28 citation statements)

References 11 publications

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“…19,20 There is no consensus about which laboratory tests and radiologic procedures should be systematically performed in patients with ES to look for an underlying disease. On the basis of this analysis and on our previous experience on isolated wAIHAs, 21 we suggest that a minimal workup, including a chest and abdominal computed tomography scan should be performed in every patient diagnosed with ES (see Table 5 for proposals). To avoid a diagnostic bias, we arbitrarily considered 10 years as being the maximum delay between both cytopenias to retain the diagnosis of ES.…”

Section: Discussionmentioning

confidence: 92%

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Michel

Chanet²,

Dechartres

et al. 2009

Blood

239

257

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Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as "primary" in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one "second-line" treatment, including splenectomy(n ‫؍‬ 19) and rituximab (n ‫؍‬ 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders. (Blood. 2009;114:3167-3172)

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Section: Discussionmentioning

confidence: 92%

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Michel

Chanet²,

Dechartres

et al. 2009

Blood

239

257

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Section: Other Optionsmentioning

confidence: 99%

“…58 In contrast, a more recent retrospective study did not observe any substantial modification in the response rate nor in the duration of prednisone therapy in patients treated with danazol. 59 No article supporting its use has been published in the last decade.Intravenous immunoglobulins (IVIG) are frequently used in AIHA, alone or in combination with prednisone, 60 and mostly in children, probably because of their proven effectiveness in primary immune thrombocytopenia, and the relatively low incidence of adverse effects compared with other treatment options. However, their use is controversial, primarily because only small case series have been reported.…”

mentioning

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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“…Usually, WAIHA patients insidiously develop anemic symptoms such as weakness, dizziness, fatigue, and dyspnea. 1,2,5,7 Typically, autoimmune anemias are macrocytic because of the presence of marked reticulocytosis, but early in the presentation, transient reticulocytopenia may occur despite the presence of a normal or hyperplastic bone marrow. Reticulocytopenia may be seen due to the in vivo hemolysis of the reticulocytes or because of compromised bone marrow function.…”

Section: Warm Autoimmune Hemolytic Anemiamentioning

confidence: 99%

Autoimmune hemolytic anemia: transfusion challenges and solutions

Barros¹,

Langhi²,

Bordin³

2017

IJCTM

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Autoimmune hemolytic anemia (AIHA) is defined as the increased destruction of red blood cells (RBCs) in the presence of anti-RBC autoantibodies and/or complement. Classification of AIHA is based on the optimal auto-RBC antibody reactivity temperatures and includes warm, cold-reactive, mixed AIHA, and drug-induced AIHA subtypes. AIHA is a rare disease, and recommendations for transfusion are based mainly on results from retrospective data and relatively small cohort studies, including heterogeneous patient samples or single case reports. In this article, we will review the challenges and solutions to safely transfuse AIHA patients. We will reflect on the indication for transfusion in AIHA and the difficulty in the accomplishment of immunohematological procedures for the selection of the safest and most compatible RBC units.

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Caractéristiques des anémies hémolytiques auto-immunes de l’adulte : analyse rétrospective d’une série de 83 patients

Cited by 76 publications

References 11 publications

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Treatment of autoimmune hemolytic anemias

Autoimmune hemolytic anemia: transfusion challenges and solutions

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