Close Menu

Books in a Library

Homozygous Sickle Cell Disease in Uganda And Jamaica: A Comparison of Bantu and Benin Haplotypes

DOI: 
Doi:10.7727/wimj.2012.167

Objective: To compare the haematological and clinical features of homozygous sickle cell (SS) disease in Bantu and Benin haplotypes in a cross-sectional study of 115 Ugandan patients attending the Sickle Cell Clinic at Mulago Hospital, Kampala, Uganda, with 311 patients in the Jamaican Cohort Study.

Methods: This involved the comparison of clinical features and haematology with special reference to genetic determinants of severity including fetal haemoglobin levels, beta-globin haplotype, and alpha thalassaemia status.

Results: The Bantu haplotype accounted for 94% of HbS chromosomes in Ugandan patients and the Benin haplotype for 76% of HbS chromosomes in Jamaica. Ugandan patients were marginally more likely to have alpha thalassaemia, had similar total haemoglobin and fetal haemoglobin levels but had higher reticulocyte counts and total bilirubin levels consistent with greater haemolysis. Ugandan patients had less leg ulceration and priapism, but the mode of clinical presentation, prevalence of dactylitis, features of bone pain and degree of delay in sexual development, assessed by menarche, were similar in the groups. In Ugandan patients, a history of anaemic episodes was common but these were poorly documented.

Conclusion: The haematological and clinical features of the Bantu haplotype in Uganda were broadly similar to the Benin haplotype in Jamaica except for less leg ulceration and priapism and possibly greater haemolysis among Ugandan subjects. Anaemic episodes in Uganda were treated empirically by transfusion often without a clear diagnosis; better documentation including reticulocyte counts and observations on spleen size is necessary to evolve appropriate models of care.

PDF Attachment: 
e-Published: 15 Feb, 2013

Pre-published Manuscript

This manuscript has been assigned to a volume and issue but has not yet been published. It is either being edited, typeset or is in the proof stage of publication.
In the pre-published stage, this manuscript may contain statements, opinions, and information that have errors in facts, figures, or interpretation. Any final changes in this manuscript will be made at the time of publication and will be reflected in the final electronic version of the issue. The editors and authors and their respective employees are not responsible or liable for the use of any such inaccurate or misleading data, opinion or information contained in the articles in this section.

Top of Page