RESUMO
Background: Sickle cell anaemia (SCA) is the most common genetic diseases in the Democratic Republic of Congo (DRC). It is estimated 30,000 to 40,000 neonates with SCA are born annually. Despite this high incidence rate, and the severity of the Bantu haplotype found in Congolese patients, major clinical characteristics remain poorly defined. The objective of this study was to assess the clinical and haematological profile of the SCA in patients less than 24 years of age. Methods: A cross-sectional study was conducted in Kinshasa, the large city of the DRC. Patients were consecutively selected in three health institutions. Results: The study includes 256 sickle cell patients. The mean age of 8.4 (SD = 4.9) years. The Hand-foot syndrome was most common (52.7%) first presentation revealing the disease in our series. The most prevalent crises found in our series were vaso-occlusive crises (VOC) in 170 cases (66.4%) and severe hemolysis in 136 cases (53.1%). Splenic sequestration was noted in 19 cases (7.4%). The age at the first pain crisis was 18.2±15.2 months-of-age and the age at the first transfusions was 29.2±27.6 months-of -age. The most common signs associated with sickle cell disease in our series were hepatomegaly (53.9%), splenomegaly (41.7%), and adenotonsillar hypertrophy (34.8%). Epistaxis was reported in 9.4%. Conclusion: The clinical course of patients in DRC was comparable to reports from Western countries, with the notable exception of epistaxis which was significantly higher in patients in the DRC.