Homozygous Delta-Beta Thalassaemia With Alpha Thalassaemia and Erythrocytosis- a Rare Case Report
In this report, we describe a case of homozygous delta-beta (δβ) thalassaemia, a rare genetic disorder characterized by severe deficiency in delta (δ) and beta (β)-globin chain production, leading to ineffective erythropoiesis and chronic haemolytic anaemia. The patient, a 26-year-old female with δβ-thalassaemia, experienced a miscarriage. High-performance liquid chromatography revealed 89.5% foetal haemoglobin (HbF) and 14.4% glycated HbF. Sebia capillary electrophoresis showed haemoglobin peak of 97.2% and 2.8%. Kleihauer Bekte test indicated a pancellular pattern of foetal cells, while morphology analysis demonstrated microcytic, hypochromic red cells and target cells. Gene analysis confirmed compound heterozygosity for two large deletions in the β-globin gene cluster.
Authors
- Mandeep Kaur Marwah (ORCID: https://orcid.org/0000-0003-4881-003X)
- Hala Shokr (ORCID: https://orcid.org/0000-0002-0973-0558)
- Hisam Siddiqi (ORCID: https://orcid.org/0009-0007-2791-838X)
- Sukhjinder Marwah (ORCID: https://orcid.org/0000-0001-8972-4723)
- Christine L. Wright
Institutions
- Aston University (GB)
- Sandwell & West Birmingham Hospitals NHS Trust (GB)
- Manchester Academic Health Science Centre (GB)
- Sandwell College (GB)
Publication Details
- Journal
- British Journal of Biomedical Science
- Published
- 2024-11-21
- DOI
- https://doi.org/10.3389/bjbs.2024.13663
- Citations
- 1
- Primary Topic
- Hemoglobinopathies and Related Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.44