Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight

Thalassaemia, a common inherited blood disorder, involves defective globin production leading to premature erythrocyte destruction. Autoimmune haemolytic anaemia (AIHA), a rare complication in thalassaemia, is characterised by autoantibody production against erythrocytes. We present a case of a 7-mo...

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Main Authors: Idris, Faridah, Abd Rahman, Siti Umairah, Mohd Tohit, Eusni Rahayu, Stephen, Claudia Cassandra, Mohamed, Rashidah, Wan Alkamar Shah, Wan Nurul Husna
Format: Article
Language:English
Published: Faculty of Medicine and Health Sciences, UPM 2024
Online Access:http://psasir.upm.edu.my/id/eprint/118152/
http://psasir.upm.edu.my/id/eprint/118152/1/118152.pdf
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author Idris, Faridah
Abd Rahman, Siti Umairah
Mohd Tohit, Eusni Rahayu
Stephen, Claudia Cassandra
Mohamed, Rashidah
Wan Alkamar Shah, Wan Nurul Husna
author_facet Idris, Faridah
Abd Rahman, Siti Umairah
Mohd Tohit, Eusni Rahayu
Stephen, Claudia Cassandra
Mohamed, Rashidah
Wan Alkamar Shah, Wan Nurul Husna
author_sort Idris, Faridah
building UPM Institutional Repository
collection Online Access
description Thalassaemia, a common inherited blood disorder, involves defective globin production leading to premature erythrocyte destruction. Autoimmune haemolytic anaemia (AIHA), a rare complication in thalassaemia, is characterised by autoantibody production against erythrocytes. We present a case of a 7-month-old boy diagnosed with β-thalassaemia major (BTM), complicated by AIHA six weeks after initiating blood transfusions. Despite daily transfusions and oral Prednisolone, the haemoglobin (Hb) level dropped to a life-threatening level (2.5g/dl). High-dose IV Methylprednisolone and Rituximab were administered, resulting in Hb improvement to 11.9 g/dL. AIHA resolution was indicated by negative C3d and reduced IgG on repeated testing. Recognition of AIHA in BTM is vital for effective management, including immunosuppressive therapy, leucodepleted transfusions, and potential splenectomy or haematopoietic stem cell transplant. Early intervention is crucial to reduce the mortality associated with AIHA in children.
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spelling upm-1181522025-06-26T02:21:53Z http://psasir.upm.edu.my/id/eprint/118152/ Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight Idris, Faridah Abd Rahman, Siti Umairah Mohd Tohit, Eusni Rahayu Stephen, Claudia Cassandra Mohamed, Rashidah Wan Alkamar Shah, Wan Nurul Husna Thalassaemia, a common inherited blood disorder, involves defective globin production leading to premature erythrocyte destruction. Autoimmune haemolytic anaemia (AIHA), a rare complication in thalassaemia, is characterised by autoantibody production against erythrocytes. We present a case of a 7-month-old boy diagnosed with β-thalassaemia major (BTM), complicated by AIHA six weeks after initiating blood transfusions. Despite daily transfusions and oral Prednisolone, the haemoglobin (Hb) level dropped to a life-threatening level (2.5g/dl). High-dose IV Methylprednisolone and Rituximab were administered, resulting in Hb improvement to 11.9 g/dL. AIHA resolution was indicated by negative C3d and reduced IgG on repeated testing. Recognition of AIHA in BTM is vital for effective management, including immunosuppressive therapy, leucodepleted transfusions, and potential splenectomy or haematopoietic stem cell transplant. Early intervention is crucial to reduce the mortality associated with AIHA in children. Faculty of Medicine and Health Sciences, UPM 2024 Article PeerReviewed text en http://psasir.upm.edu.my/id/eprint/118152/1/118152.pdf Idris, Faridah and Abd Rahman, Siti Umairah and Mohd Tohit, Eusni Rahayu and Stephen, Claudia Cassandra and Mohamed, Rashidah and Wan Alkamar Shah, Wan Nurul Husna (2024) Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight. Malaysian Journal of Medicine and Health Sciences, 20 (suppl.11). pp. 88-91. ISSN 1675-8544 https://medic.upm.edu.my/upload/dokumen/2024123018293516_MJMHS_0412.pdf 10.47836/mjmhs.20.s11.16
spellingShingle Idris, Faridah
Abd Rahman, Siti Umairah
Mohd Tohit, Eusni Rahayu
Stephen, Claudia Cassandra
Mohamed, Rashidah
Wan Alkamar Shah, Wan Nurul Husna
Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title_full Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title_fullStr Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title_full_unstemmed Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title_short Co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
title_sort co-occurrence of autoimmune haemolytic anaemia and β-thalassaemia major in an infant: a case report and updated pathogenic insight
url http://psasir.upm.edu.my/id/eprint/118152/
http://psasir.upm.edu.my/id/eprint/118152/
http://psasir.upm.edu.my/id/eprint/118152/
http://psasir.upm.edu.my/id/eprint/118152/1/118152.pdf