Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders

Background/Aims: Individuals with double heterozygosity for alpha- and beta-thalassaemia and heterozygous beta-thalassaemia show a similar haematological picture. Co-inheritance of (alpha- and beta-thalassaemia in both partners may result in pregnancies with either Hb Bart's hydrops foetalis or...

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Main Authors: Wee, Y.C., Tan, K.L., Kuldip, K., Tai, K.S., George, E., Tan, P.C., Chia, P., Subramaniam, R., Yap, S.F., Tan, J.
Format: Article
Language:English
Published: 2008
Subjects:
Online Access:http://eprints.um.edu.my/3715/1/Alpha-thalassaemia_in_association_with_beta-thalassaemia_patients.pdf
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author Wee, Y.C.
Tan, K.L.
Kuldip, K.
Tai, K.S.
George, E.
Tan, P.C.
Chia, P.
Subramaniam, R.
Yap, S.F.
Tan, J.
author_facet Wee, Y.C.
Tan, K.L.
Kuldip, K.
Tai, K.S.
George, E.
Tan, P.C.
Chia, P.
Subramaniam, R.
Yap, S.F.
Tan, J.
author_sort Wee, Y.C.
collection UM
description Background/Aims: Individuals with double heterozygosity for alpha- and beta-thalassaemia and heterozygous beta-thalassaemia show a similar haematological picture. Co-inheritance of (alpha- and beta-thalassaemia in both partners may result in pregnancies with either Hb Bart's hydrops foetalis or beta-thalassaemia major, or pregnancies with both disorders. Methods: The co-inheritance of a-thalassaemia in 322 P-thalassaemia carriers in Malaysia was studied. Results: The frequency of (alpha-thalassaemia in the beta-thalassaemia carriers was 12.7 (41/322), with a carrier frequency of 7.8 for the SEA deletion, 3.7 for the -alpha(3.7) deletion, 0.9 for Hb Constant Spring and 0.3 for the -alpha(4.2) deletion. Conclusion: Double heterozygosity for (alpha- and beta-thalassaemia was confirmed in 5 out of the 41 couples and the risk of the fatal condition Hb Bart's hydrops foetalis was confirmed in two of these couples. Detection of the Southeast Asian (SEA) deletion in the Malaysian Malays in this study confirms that Hb Bart's hydrops foetalis can occur in this ethnic group. Results of this study have provided new information on the frequency and different types of alpha-thalassaemia (--(SEA), -alpha(3.7) and -alpha(4-1) deletions, Hb Constant Spring) in Malaysian beta-thalassaemia carriers.Copyright (C) 2008 S. Karger AG, Basel.
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spelling um.eprints-37152012-10-04T00:52:02Z http://eprints.um.edu.my/3715/ Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders Wee, Y.C. Tan, K.L. Kuldip, K. Tai, K.S. George, E. Tan, P.C. Chia, P. Subramaniam, R. Yap, S.F. Tan, J. R Medicine Background/Aims: Individuals with double heterozygosity for alpha- and beta-thalassaemia and heterozygous beta-thalassaemia show a similar haematological picture. Co-inheritance of (alpha- and beta-thalassaemia in both partners may result in pregnancies with either Hb Bart's hydrops foetalis or beta-thalassaemia major, or pregnancies with both disorders. Methods: The co-inheritance of a-thalassaemia in 322 P-thalassaemia carriers in Malaysia was studied. Results: The frequency of (alpha-thalassaemia in the beta-thalassaemia carriers was 12.7 (41/322), with a carrier frequency of 7.8 for the SEA deletion, 3.7 for the -alpha(3.7) deletion, 0.9 for Hb Constant Spring and 0.3 for the -alpha(4.2) deletion. Conclusion: Double heterozygosity for (alpha- and beta-thalassaemia was confirmed in 5 out of the 41 couples and the risk of the fatal condition Hb Bart's hydrops foetalis was confirmed in two of these couples. Detection of the Southeast Asian (SEA) deletion in the Malaysian Malays in this study confirms that Hb Bart's hydrops foetalis can occur in this ethnic group. Results of this study have provided new information on the frequency and different types of alpha-thalassaemia (--(SEA), -alpha(3.7) and -alpha(4-1) deletions, Hb Constant Spring) in Malaysian beta-thalassaemia carriers.Copyright (C) 2008 S. Karger AG, Basel. 2008 Article PeerReviewed application/pdf en http://eprints.um.edu.my/3715/1/Alpha-thalassaemia_in_association_with_beta-thalassaemia_patients.pdf Wee, Y.C. and Tan, K.L. and Kuldip, K. and Tai, K.S. and George, E. and Tan, P.C. and Chia, P. and Subramaniam, R. and Yap, S.F. and Tan, J. (2008) Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders. Community Genetics, 11 (3). pp. 129-134. ISSN 1422-2795, DOI https://doi.org/10.1159/000113874 <https://doi.org/10.1159/000113874>. 10.1159/000113874
spellingShingle R Medicine
Wee, Y.C.
Tan, K.L.
Kuldip, K.
Tai, K.S.
George, E.
Tan, P.C.
Chia, P.
Subramaniam, R.
Yap, S.F.
Tan, J.
Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title_full Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title_fullStr Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title_full_unstemmed Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title_short Alpha-thalassaemia in association with beta-thalassaemia patients in Malaysia: a study on the co-inheritance of both disorders
title_sort alpha thalassaemia in association with beta thalassaemia patients in malaysia a study on the co inheritance of both disorders
topic R Medicine
url http://eprints.um.edu.my/3715/1/Alpha-thalassaemia_in_association_with_beta-thalassaemia_patients.pdf
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