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

Abstract

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

Plasmapheresis in a child with cold antibody autoimmune hemolytic anemia: case report

“…In a study by Aladjidi et al, (5) in which 265 children with AIHA were evaluated retrospectively, it was shown that 37% of patients had primary AIHA, 63% had secondary AIHA, previous infection was present in 10% of the cases of secondary AIHA, and immunologic causes were present in 53%. It has been shown that immunologic diseases develop subsequently in approximately half of patients who report infection initially and these patients show a chronic prognosis.…”

Coomb’s Test Negative Autoimmune Hemolytic Anemia

“…These serological tests are considered important to exclude AIHA secondary to these infections. 2 AIHA secondary to infections was considered less likely in this patient due to compatible clinical picture and recurrent acute hemolysis. 2 There was unusual Þ nding of Vitamin B12 deÞ ciency in our patient.…”

“…2 AIHA secondary to infections was considered less likely in this patient due to compatible clinical picture and recurrent acute hemolysis. 2 There was unusual Þ nding of Vitamin B12 deÞ ciency in our patient. Despite treatment with Vitamin B12, there was no clinical improvement and only after initiation of oral prednisolone, hemolytic activity was reduced thus obviating need for further transfusions.…”

“…1 The diagnosis of AIHA is based on hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test (DAT) and at least one of the following features of hemolysis: reticulocyte count greater than 120×10 9 /L, haptoglobin less than 10 mg/dL, and total bilirubin greater than 1 mg/dL. 2 A positive DAT suggests diagnosis of AIHA however DAT negative AIHA occurs in 5-10% of patients.…”

“…1 These are uncommon causes of anemia in children 1,2 and should be considered in a differential diagnosis of acquired hemolytic anemia or secondary manifestations of autoimmune diseases, lymphoproliferative disease and viral or mycoplasma infections. 1 Here, we report a case of a 14 year-old girl with Coomb's negative autoimmune hemolytic anemia which responded with prednisolone and had lab evidence of Vitamin B12 deÞ ciency.…”