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

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Title: New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children
Authors: Nathalie Aladjidi, Guy Leverger, Thierry Leblanc, Marie Quitterie Picat, Gérard Michel, Yves Bertrand, Brigitte Bader-Meunier, Alain Robert, Brigitte Nelken, Virginie Gandemer, Hélène Savel, Jean Louis Stephan, Fanny Fouyssac, Julien Jeanpetit, Caroline Thomas, Pierre Rohrlich, André Baruchel, Alain Fischer, Geneviève Chêne, Y. Perel
Source: Haematologica, Vol 96, Iss 5 (2011)
Publisher Information: Ferrata Storti Foundation, 2011.
Publication Year: 2011
Collection: LCC:Diseases of the blood and blood-forming organs
Subject Terms: Diseases of the blood and blood-forming organs, RC633-647.5
More Details: Background Autoimmune hemolytic anemia is a rare condition in children. Little is known about its initial presentation and the subsequent progression of the disease.Design and Methods 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.Results The 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).Conclusions This 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.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 0390-6078
1592-8721
Relation: https://haematologica.org/article/view/5960; https://doaj.org/toc/0390-6078; https://doaj.org/toc/1592-8721
DOI: 10.3324/haematol.2010.036053
Access URL: https://doaj.org/article/74a00a33c1c6499b8ed73af5850fcd76
Accession Number: edsdoj.74a00a33c1c6499b8ed73af5850fcd76
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  Data: Background Autoimmune hemolytic anemia is a rare condition in children. Little is known about its initial presentation and the subsequent progression of the disease.Design and Methods 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.Results The 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).Conclusions This 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.
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  Data: https://haematologica.org/article/view/5960; https://doaj.org/toc/0390-6078; https://doaj.org/toc/1592-8721
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