Impact of Haplotypes on the Frequency of Morbid Complications in Homozygous SSFA2 Sickle Cell Disease in Cote D’ivoire

Abstract

Background: Sickle cell disease is a constitutional hemoglobin disease witch poses a public health problem in Côte d’Ivoire due to its prevalence and complications. The homozygous form (SSFA2) is the most severe. The proportion of hemoglobin F by its property determines the haplotype. Authors wanted to determine the impact of these haplotypes on the frequency of morbid complications. Methods: Our study was a transversal type and analytical aims, occurred in the clinical hematology department of the University Hospital of Yopougon over a 3 months period. Our study included 150 SSFA2 patients with complications. The statistical test used was the student. Results: The mean age was 11 years, (6 months to 42 years). The sex ratio was 1.05. The mean rate of hemoglobin S was 86%, of which 17% had severe haplotype, 37% intermediate haplotype, and 45% benign haplotype. Infectious complications were the most frequent (58.72%) (Malaria 53.47%; bronchial pneumonia: 28.22%), followed by anemic complications (36.92%) and ischemic complications (4.36%). Deglobulization crisis was the major acute anemic complication (97.5%) followed by splenic sequestration (2.5%). Chronic anemic complications were dominated by leg ulcers (57.14%) followed by biliary lithiasis (42.86%). Aseptic necrosis of the femoral head was the most frequent ischemic complication (46.66%), followed by retinopathy (33.33%), and then stroke (20%). The severe haplotype was associated with a high frequency of complications in general and infectious complications in particular. (P=0.005) Conclusion: The clinical expression of the SSFA2 homozygous form and the occurrence of complications is closely related to the haplotype.