Tite M Mikobi1, Prosper Lukusa Tshilobo2, Michel N Aloni3, Pierre Z Akilimali4, Georges Mvumbi-Lelo1, Jean Marie Mbuyi-Muamba5. 1. Department des Sciences de Bases, Laboratory of Biochemistry and Molecular Biology, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo. 2. Unit of Human Genetics, Department of Paediatrics, Faculty of Medicine, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Democratic Republic of Congo. 3. Division of Hemato-oncology and Nephrology, Department of Paediatrics, University Hospital of Kinshasa, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo. 4. Division of Biostatistics and Epidemiology, School of Public Health, University of Kinshasa, Kinshasa, Democratic Republic of Congo. 5. Division of Hemato-Immuno-rhumatology, Department of Internal Medicine, Faculty of Medicine, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
Abstract
BACKGROUND: The influence of phenotype on the clinical course and laboratory features of sickle cell anemia (SCA) is rarely described in sub-Saharan Africa. METHODS: A cross-sectional study was conducted in Kinshasa. A clinical phenotype score was built up. The following definitions were applied: asymptomatic clinical phenotype (ACP; score≤5), moderate clinical phenotype (MCP; score between 6 and 15), and severe clinical phenotype (SCP; score≥16). ANOVA test were used to compare differences among categorical variables. RESULTS: We have studied 140 patients. The mean body mass index (BMI) value of three groups was lower (<25 kg/m2 ) than the limit defining overweight. BMI of the subjects with ACP was significantly higher than those of other phenotypes (P<.05). Sickle cell patients with ACP have a high mean steady-state hemoglobin concentration compared to those with MCP and SCP (P<.001). A significant elevated baseline leukocyte count is associated with SCP (P<.001). Fetal Hemoglobin (HbF) was significantly higher in ACP. Significant elevation of alpha 1 and alpha 2 globulins in SCP were observed. CONCLUSION: In our study, fetal hemoglobin has an influence on the clinical severity and the biological parameters of SCA. The study provides data concerning the sickle cell anemia clinical and biological variability in our midst.
BACKGROUND: The influence of phenotype on the clinical course and laboratory features of sickle cell anemia (SCA) is rarely described in sub-Saharan Africa. METHODS: A cross-sectional study was conducted in Kinshasa. A clinical phenotype score was built up. The following definitions were applied: asymptomatic clinical phenotype (ACP; score≤5), moderate clinical phenotype (MCP; score between 6 and 15), and severe clinical phenotype (SCP; score≥16). ANOVA test were used to compare differences among categorical variables. RESULTS: We have studied 140 patients. The mean body mass index (BMI) value of three groups was lower (<25 kg/m2 ) than the limit defining overweight. BMI of the subjects with ACP was significantly higher than those of other phenotypes (P<.05). Sickle cell patients with ACP have a high mean steady-state hemoglobin concentration compared to those with MCP and SCP (P<.001). A significant elevated baseline leukocyte count is associated with SCP (P<.001). Fetal Hemoglobin (HbF) was significantly higher in ACP. Significant elevation of alpha 1 and alpha 2 globulins in SCP were observed. CONCLUSION: In our study, fetal hemoglobin has an influence on the clinical severity and the biological parameters of SCA. The study provides data concerning the sickle cell anemia clinical and biological variability in our midst.
Authors: S T Miller; L A Sleeper; C H Pegelow; L E Enos; W C Wang; S J Weiner; D L Wethers; J Smith; T R Kinney Journal: N Engl J Med Date: 2000-01-13 Impact factor: 91.245
Authors: Andreia A Canalli; Renata F Proença; Carla F Franco-Penteado; Fabiola Traina; Tatiana M Sakamoto; Sara T O Saad; Nicola Conran; Fernando F Costa Journal: Haematologica Date: 2010-12-20 Impact factor: 9.941