Autoimmune Hemolytic Anemia in Children

Naithani, Rahul; Agrawal, Neha; Mahapatra, Manoranjan; Kumar, Rajat; Pati, Hara Prasad; Choudhry, V. P.

doi:10.1080/08880010701360783

Cited by 60 publications

(61 citation statements)

References 9 publications

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Section: Corticosteroidsmentioning

confidence: 99%

“…18 Moreover, an earlier onset of steroid therapy correlates with a lower probability of relapse. 16 It is worth remembering that AIHA patients on prolonged steroid therapy should be given bisphosphonates, vitamin D, calcium, and folic acid supplementation.2 Patients with particularly rapid hemolysis and very severe anemia, or complex cases such as Evans syndrome, may require intravenous methylprednisolone at 100-200 mg/day for 10-14 days or 250-1000 mg/day for 1-3 days, although highdose corticosteroid therapy for AIHA has been described essentially as case reports. 19,20 First-line therapy with corticosteroids is expected to provide a response in 70-85% of patients; however, only 1 in 3 cases remain in longterm remission once the drug is discontinued, a further 50% require maintenance doses, and approximately 20-30% need additional second-line therapies.…”

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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: Corticosteroidsmentioning

confidence: 99%

mentioning

confidence: 99%

Treatment of autoimmune hemolytic anemias

2014

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“…[2][3][4][5][6] Recent literature contains information from small series, mainly based on laboratory tests, and involving very few centers. [7][8][9][10] The prevalence of AIHA in childhood is still unknown, but likely increases with age, as for most autoimmune disorders. Evans' syndrome (ES) was first described as hemolytic anemia with a positive direct antiglobulin test (DAT) and immune thrombocytopenia occurring simultaneously or in succession, in the absence of any known etiology.…”

Section: Introductionmentioning

confidence: 99%

New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children

Aladjidi¹,

Leverger²,

Leblanc³

et al. 2011

Haematologica

191

180

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Since 2004, a national observational study has been aiming to thoroughly describe cases and identify prognostic factors. Patients from all French hematologic pediatric units have been included if they had a hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test and hemolysis. Evans' syndrome was defined by the association of autoimmune hemolytic anemia and immunological thrombocytopenic purpura. Data from patients' medical records were registered from birth to last follow-up. Autoimmune hemolytic anemia was classified as primary or secondary. Remission criteria, qualifying the status of anemia at last follow-up, were used with the aim of identifying a subgroup with a favorable prognosis in continuous complete remission. ResultsThe first 265 patients had a median age of 3.8 years at diagnosis. In 74% of cases the direct antiglobulin test was IgG/IgG+C3d. Consanguinity was reported in 8% of cases and first degree familial immunological diseases in 15% of cases. Evans' syndrome was diagnosed in 37% of cases. Autoimmune hemolytic anemia was post-infectious in 10%, immunological in 53% and primary in 37% of cases. After a median follow-up of 3 years, 4% of children had died, 28% were still treatment-dependent and 39% were in continuous complete remission. In multivariate analysis, IgG and IgG+C3d direct antiglobulin tests were associated with a lower rate of survival with continuous complete remission (adjusted hazard ratio, 0.43; 95% confidence interval, 0.21-0.86). ConclusionsThis nationwide French cohort is the largest reported study of childhood autoimmune hemolytic anemia. The rarity of this condition is confirmed. Subgroups with genetic predisposition and underlying immune disorders were identified.Key words: autoimmune hemolytic anemia, children, prognostic factors, Evans' syndrome, rare diseases. Citation: Aladjidi N, Leverger G, Leblanc T, Picat MQ, Michel G, Bertrand Y, Bader-Meunier

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“…Some steroid-dependent patients, such as patient 2, may require maintenance with low-dose corticosteroids for an extended period [13]. In patients dependent on high daily doses (prednisolone >0.5 mg/kg) or in corticosteroid-refractory patients, cyclosporine should be tried before rituximab, cyclophosphamide, or splenectomy [5]. In the present study 1 patient with primary AIHA and 1 with primary ES required CsA.…”

Section: Discussionmentioning

confidence: 99%

“…Childhood AIHA often presents as an acute selflimited illness, with good response to short-term steroid therapy in 80% of the patients [5]. In infants and adolescents onset can be insidious, with a tendency to become chronic [6].…”

Section: Introductionmentioning

confidence: 99%

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

Sarper¹,

Kılıç²,

Zengin³

et al. 2011

Turk J Hematol

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Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA). Materials and Methods: The medical records of all patients (n=19) diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed. Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years). In all, 13 patients had primary (idiopathic) AIHA, whereas 2 had primary Evans Syndrome (ES), 2 had autoimmune lymphoproliferative syndrome (ALPS)+ES, and 1 had Wiskott-Aldrich syndrome (WAS)+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years) at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia. Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS. (Turk J Hematol 2011; 28: 198-205)

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Autoimmune Hemolytic Anemia in Children

Cited by 60 publications

References 9 publications

Treatment of autoimmune hemolytic anemias

Treatment of autoimmune hemolytic anemias

New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

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