Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome

BACKGROUND: Autoimmune hemolytic anemias (AIHA) are characterized by the destruction of red cells following the production of autoantibodies directed against them. Although AIHA in children is usually self-limiting, many still succumb to the illness due to delay in the diagnosis and treatment. AIHA...

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Autores principales:	Satyam Arora, Seema Dua, Nita Radhakrishnan, Savitri Singh, Jyotsna Madan, Devajit Nath
Formato:	article
Lenguaje:	EN
Publicado:	Wolters Kluwer Medknow Publications 2021
Materias:	autoimmune direct coomb's test hemolytic anemia Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/3e58b49f2cca440dbaa785dfe30ed5e6
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spelling	oai:doaj.org-article:3e58b49f2cca440dbaa785dfe30ed5e62021-11-12T10:08:28ZAutoimmune hemolytic anemia in children: Clinical presentation and treatment outcome0973-62471998-356510.4103/ajts.AJTS_31_20https://doaj.org/article/3e58b49f2cca440dbaa785dfe30ed5e62021-01-01T00:00:00Zhttp://www.ajts.org/article.asp?issn=0973-6247;year=2021;volume=15;issue=2;spage=160;epage=165;aulast=Arorahttps://doaj.org/toc/0973-6247https://doaj.org/toc/1998-3565BACKGROUND: Autoimmune hemolytic anemias (AIHA) are characterized by the destruction of red cells following the production of autoantibodies directed against them. Although AIHA in children is usually self-limiting, many still succumb to the illness due to delay in the diagnosis and treatment. AIHA in children may be secondary to autoimmune diseases, drugs, or immune deficiencies. Early diagnosis and appropriate immunohematological evaluation can aid in the diagnosis and treatment.OBJECTIVE: To analyze the evaluation, treatment, and outcome of AIHA in children.METHODS: Prospective data of patients aged 0–18 years diagnosed with AIHA between June 2017 and May 2019 were collected.INTERVENTION: Prednisolone was the first-line agent in all; second-line agents included cyclosporine and rituximab. Red cell transfusion was given in those with severe anemia with cardiac decompensation.RESULTS: Eleven patients were diagnosed during the study period. Hemoglobin ranged from 1.2 to 9 g/dl. The initial presentation was severe anemia in 8 children and moderate anemia with thrombocytopenia in 3. The trigger was infection in 5. Polyspecific direct coomb's test (DCT) was positive in 10 patients. 2/10 polyspecific DCT-positive cases on further evaluation had immunoglobulin G (IgG) and C3d positivity, whereas rest 8 had only IgG. One infant was diagnosed with DCT-negative immunoglobulin A-mediated AIHA. 4/11 attained remission following the short course of prednisolone. Cyclosporine was used as the second-line agent in 2 and rituximab was used in 3. Seven children are in sustained remission and off medication. One died within 12 h of diagnosis.CONCLUSION: AIHA is not an uncommon problem in children and can vary in its clinical severity. Early and correct diagnosis helps in deciding appropriate treatment.Satyam AroraSeema DuaNita RadhakrishnanSavitri SinghJyotsna MadanDevajit NathWolters Kluwer Medknow Publicationsarticleautoimmunedirect coomb's testhemolytic anemiaDiseases of the blood and blood-forming organsRC633-647.5ENAsian Journal of Transfusion Science, Vol 15, Iss 2, Pp 160-165 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	autoimmune direct coomb's test hemolytic anemia Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	autoimmune direct coomb's test hemolytic anemia Diseases of the blood and blood-forming organs RC633-647.5 Satyam Arora Seema Dua Nita Radhakrishnan Savitri Singh Jyotsna Madan Devajit Nath Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
description	BACKGROUND: Autoimmune hemolytic anemias (AIHA) are characterized by the destruction of red cells following the production of autoantibodies directed against them. Although AIHA in children is usually self-limiting, many still succumb to the illness due to delay in the diagnosis and treatment. AIHA in children may be secondary to autoimmune diseases, drugs, or immune deficiencies. Early diagnosis and appropriate immunohematological evaluation can aid in the diagnosis and treatment.OBJECTIVE: To analyze the evaluation, treatment, and outcome of AIHA in children.METHODS: Prospective data of patients aged 0–18 years diagnosed with AIHA between June 2017 and May 2019 were collected.INTERVENTION: Prednisolone was the first-line agent in all; second-line agents included cyclosporine and rituximab. Red cell transfusion was given in those with severe anemia with cardiac decompensation.RESULTS: Eleven patients were diagnosed during the study period. Hemoglobin ranged from 1.2 to 9 g/dl. The initial presentation was severe anemia in 8 children and moderate anemia with thrombocytopenia in 3. The trigger was infection in 5. Polyspecific direct coomb's test (DCT) was positive in 10 patients. 2/10 polyspecific DCT-positive cases on further evaluation had immunoglobulin G (IgG) and C3d positivity, whereas rest 8 had only IgG. One infant was diagnosed with DCT-negative immunoglobulin A-mediated AIHA. 4/11 attained remission following the short course of prednisolone. Cyclosporine was used as the second-line agent in 2 and rituximab was used in 3. Seven children are in sustained remission and off medication. One died within 12 h of diagnosis.CONCLUSION: AIHA is not an uncommon problem in children and can vary in its clinical severity. Early and correct diagnosis helps in deciding appropriate treatment.
format	article
author	Satyam Arora Seema Dua Nita Radhakrishnan Savitri Singh Jyotsna Madan Devajit Nath
author_facet	Satyam Arora Seema Dua Nita Radhakrishnan Savitri Singh Jyotsna Madan Devajit Nath
author_sort	Satyam Arora
title	Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
title_short	Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
title_full	Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
title_fullStr	Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
title_full_unstemmed	Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome
title_sort	autoimmune hemolytic anemia in children: clinical presentation and treatment outcome
publisher	Wolters Kluwer Medknow Publications
publishDate	2021
url	https://doaj.org/article/3e58b49f2cca440dbaa785dfe30ed5e6
work_keys_str_mv	AT satyamarora autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome AT seemadua autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome AT nitaradhakrishnan autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome AT savitrisingh autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome AT jyotsnamadan autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome AT devajitnath autoimmunehemolyticanemiainchildrenclinicalpresentationandtreatmentoutcome
_version_	1718431043005972480

Autoimmune hemolytic anemia in children: Clinical presentation and treatment outcome

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