Abstract
Autoimmune Hemolytic Anemia (AIHA) in childhood is uncommon and estimated to be three
per million annually under 18 years of age. Detailed immunohematological and clinical
characterizations are essential for correct diagnosis of the disease and its management.
In this study we described AIHA in children with regards to patient demography, underlying
etiology, disease classification, antibody characterization, clinical features, degree
of in vivo hemolysis and transfusion management. The prospective observational study
was conducted over a period of 6 years and included 29 children with newly diagnosed
AIHA. Patient details were obtained from the hospital information system and patient
treatment file. The median age of the children was 12 years with a female preponderance.
Secondary AIHA was observed in 62.1% patients. The mean hemoglobin and reticulocyte
were 7.1 gm/dL and 8.8 percentages respectively. The median polyspecific direct antiglobulin
test (DAT) grading was 3+. Red cell bound multiple autoantibodies were found in 27.6%
children. Free serum autoantibodies were present in 62.1% patients. Twenty six of
the 42 units transfused were “best match” or “least incompatible”. Follow-up of 21
children showed clinical and laboratory improvement with DAT still positive at the
end of 9 months. AIHA in childhood requires advanced and efficient clinical, immunohematological
and transfusion support. Detailed characterization of AIHA is important, as they determine
degree of in vivo hemolysis, disease severity, serological incompatibility and necessity
of blood transfusion. Although blood transfusion in AIHA is a challenge but it should
not be withheld in critically ill patients.
Keywords
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References
- Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed.,. published by W.B Saunders Company Philadelphia,, USA2007
- Autoimmune hemolytic anemia.Am J Hematol. 2002; 69: 258-271
- Autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria.in: Simon T.L. McCollough J. Synder E.L. Solheim B.G. Strauss R.G. Rossi’s principles of transfusion medicine. Wiley-Blackwell publication, New Jersey, USA. 2016: 144-158
- New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children.Haematologica. 2011; 96: 655-663
- Autoimmune hemolytic anemia with warm antibodies in children: retrospective analysis of 51 cases.Turk J Pedia. 1999; 41: 467-471
- Autoimmune hemolytic anemia in children.Pedia Hematol Oncol. 2007; 24: 309-315
- Clinical course of autoimmune hemolytic anemia: an observational study.J Pedia (Rio J). 2006; 82: 58-62
- Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome.Asian J Transfus Sci. 2021; 15: 160-165
- Autoimmune haemolysis in childhood and adolescence.Acta Haematol. 1984; 72: 45-257
- Evans syndrome: results of a national survey.J Pedia Hematol Oncol. 1997; 19: 433-437
- The spectrum of Evans’ syndrome.Arch Dis Child. 1997; 77: 245-248
- Clinical Features and Treatment Outcomes of Childhood Autoimmune Hemolytic Anemia: A Retrospective Analysis of 68 Cases.J Pedia Hematol Oncol. 2016; 38 (Mar;): e50-5https://doi.org/10.1097/MPH.0000000000000476
- The acute and transient nature of idiopathic immune hemolytic anemia in childhood.J Pedia. 1976; 88: 780-783
- Investigation of a positive DAT result.Technical manual. 18th ed..,. American Association of Blood Banks,, Bethesda, Maryland2018
- The positive direct antiglobulin test and immune mediated hemolysis.Technical manual. 18th ed..,. American Association of Blood Banks,, Bethesda, Maryland2018: 425-448
- Incompatible blood transfusion: Challenging yet life saving in the management of acute severe autoimmune hemolytic anemia.Asian J Transfus Sci. 2014; 8: 1-4
- Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association.Blood Transfus. 2017; 15: 259-267https://doi.org/10.2450/2016.0072-16
Product inserts; Cat-004041, Cat-004851, Cat-004043V, Cat-004033V, BIO-RAD /DiaMed GmbH, 1785 Cressier s/Morat, Switzerland.
- Clinical and serological characterization of cold agglutinin syndrome in a Tertiary Care Hospital in Eastern India.Asian J Transfus Sci. 2015; 9: 173-176
- Warm reactive autoantibodies: Clinical and serological correlations.Am J Clin Pathol. 2004; 122: 680-685
- Epidemiology of autoimmune hemolytic anemia: A nationwide population-based study in France.Am J Hematol. 2021; 96 (10.1002/ajh.26213. Epub 2021 May 22. PMID: 33930203): E291-E293
- Clinical aspects of autoimmune hemolytic anemia.Semin Hematol. 1976; 13: 251-265
- Immunohematological characterization of DAT negative autoimmune hemolytic anemia through simple, sensitive methods: Experience from a tertiary care hospital in India.Glob J Transfus Med. 2020; 5: 197-201
- Red cell bound immunoglobulin as a predictor of severity of hemolysis in patients with autoimmune hemolytic anemia.Transfusion. 1990; 30: 576-578
- Serological diagnosis and characterization of causative antibody.in: Hugh Chaplin J.R. Churchill Livingston U.S.A. Methods in Hematology- Immune Hemolytic Anemia. 1985
- Clinical and serological characterization of autoimmune hemolytic anemia in a tertiary care hospital in North India.Ann Hematol. 2009; 88: 727-732
- Red cell autoantibodies, multiple immunoglobulin classes, and autoimmune hemolysis.Transfusion. 1990; 30: 714-717
- The value of gel test and ELAT in autoimmune hemolytic anemia. Clin.Lab Hematol. 1995; 17: 311-316
- The autoimmune hemolytic anemias.Br J Hematol. 1972; 23: 61-69
- The pathology of autoimmune hemolytic anemia.J Clin Pathol. 1992; 45: 1047-1052
- Immune Hemolytic Anemias. 2nd edition.,. Churchill Livingstone, Philadelphia, PA, USA2004
- Autoantibody formation after alloimmunization: are blood transfusions a risk factor for autoimmune hemolytic anemia?.Transfusion. 2004; 44: 67-72
- Immunohematological and clinical characterization of complement and non-complement associated warm autoimmune haemolytic anemia and risk factors predicting their occurrences.Indian J Hematol Blood Transfus. 2021; 37: 623-631
- Utility of adsorption techniques in serological evaluation of AIHA in a hospital based transfusion service.Blood Transfus. 2009; 7: 300-304
- Study on blood ABO typing in patients with autoimmune hemolytic anemia.Zhongguo shi Yan Xue Ye Xue Za Zhi. 2004; 12: 525-527
- Prophylactic antigen-matched donor blood for patients with warm autoantibodies: an algorithm for transfusion management.Transfusion. 2002; 42: 1435-1441
- Monocyte receptors for the Fc portion of IgG increased in number in autoimmune hemolytic anemia and other hemolytic states and are decreased by glucocorticoid therapy.J Immunol. 1983; 131: 1240-1245
- Autoimmune hemolytic anemia: current concepts.Aust NZ J Med. 1988; 18: 625-637
Article info
Publication history
Published online: March 14, 2023
Accepted:
March 13,
2023
Received in revised form:
February 17,
2023
Received:
December 7,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2023 Published by Elsevier Ltd.
