Current Approaches for the Treatment of Autoimmune Hemolytic Anemia

Jaime‐Pérez, José Carlos; Rodríguez-Martínez, Marisol; Gómez-De-León, Andrés; Tarín‐Arzaga, Luz; Gómez‐Almaguer, David

doi:10.1007/s00005-013-0232-3

Cited by 27 publications

(32 citation statements)

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Section: Splenectomymentioning

confidence: 99%

Section: "Last Option" Treatmentsmentioning

confidence: 99%

“…Moreover, a substantial number of them remain in remission for years without medication, with a presumed cure rate of up to 20%. 2,22,23 It is worth mentioning that patients with persistent or recurrent hemolysis after splenectomy often require lower doses of corticosteroids than before surgery.2 A drawback of splenectomy is the lack of reliable predictors of the outcome, since its effectiveness is not related to disease duration, response to steroids nor the extent of splenic sequestration. 24 Moreover, splenectomy may be associated with surgical complications (pulmonary embolism, intra-abdominal bleeding, abdominal abscess, abdominal wall hematoma), although laparoscopic intervention has lowered the surgical risk compared to conventional surgery (0.5-1.6% vs. 6%).…”

mentioning

confidence: 99%

“…It has been suggested that this drug could be included in the treatment arsenal of refractory immune cytopenias, as a steroid-sparing option. 23 Recently, mycophenolate mofetil has also been successfully used in association with rituximab in a case of post-hematopoietic stem cell transplant, refractory AIHA. …”

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confidence: 99%

“…67 Alemtuzumab, a humanized anti-CD52 monoclonal antibody, has been shown to be effective in small series of patients with idiopathic refractory AIHA, with an overall complete remission rate in 13 of 16, including 3 pediatric cases. 23,68,69 However, because of the high toxicity, it is considered a "last resort" option in severe idiopathic AIHA unresponsive to all previous treatments.2 Alemtuzumab induced an overall response in 11 of 12 cases with CLLassociated AIHA, refractory to corticosteroid, splenectomy and rituximab, suggesting that it should be considered even before rituximab in warm AIHA accompanied by progressive CLL. 22,[70][71][72] Ofatumumab, a monoclonal antibody targeting a unique epitope on CD20 that differs from that targeted by rituximab, has recently been successfully used in a case of CLL-associated warm AIHA refractory to rituximab.…”

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confidence: 99%

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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: Splenectomymentioning

confidence: 99%