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Clinical phenotypes and the biological parameters of Congolese patients suffering from sickle cell anemia: A first report from Central Africa
Author(s) -
Mikobi Tite M.,
Lukusa Tshilobo Prosper,
Aloni Michel N.,
Akilimali Pierre Z.,
MvumbiLelo Georges,
MbuyiMuamba Jean Marie
Publication year - 2017
Publication title -
journal of clinical laboratory analysis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.536
H-Index - 50
eISSN - 1098-2825
pISSN - 0887-8013
DOI - 10.1002/jcla.22140
Subject(s) - medicine , fetal hemoglobin , asymptomatic , anemia , hemoglobin , phenotype , sickle cell anemia , body mass index , gastroenterology , immunology , fetus , pregnancy , biology , disease , genetics , gene , biochemistry
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/m 2 ) 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.

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