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Early detection of sickle cell disease is crucial to improve people's survival. Both financial and geographic accessibility to sickle cell disease tools are barriers to universal screening in developing countries. The purpose of this study was to determine the hospital prevalence of sickle cell disease and to assess the reliability of a rapid diagnostic tool, HemoTypeSC, in a resource-limited environment. We conducted a prospective cross-sectional descriptive study in the Department of Pediatrics of 5 health facilities in the city of Kindu, Maniema province, DRC, over a period of 10 months. The study consisted of HemoType SC rapid test screening for sickle cell disease and then diagnostic confirmation by hemoglobin electrophoresis. A total of 448 children less than 5 years of age were enrolled in the study. The overall hospital prevalence of patients with sickle cell disease was 31.9%, of whom 12.7% were homozygous (SS) and 19.2% trait carriers; the level of suspicion for sickle cell disease in hospitals was 6%; the clinical presumption regarding sickle cell disease was 8%; HemoType SC rapid test had good indicators of validity for the detection of hemoglobins A and S. The study shows that the hospital prevalence of major sickle cell disease is higher in children under 5 years of age with respect to clinical suspicion in the absence of laboratory tests. HemoTypeSC rapid test seems to be a reliable tool for the screening of the disease in the city of Kindu, a resource-limited environment. [ABSTRACT FROM AUTHOR]

The therapeutic management of sickle cell disease is based on several strategies, in which red blood cell transfusion plays an essential role in acute complications such as vaso-occlusive crisis, acute chest syndrome and stroke. However, it is important to weigh the benefit/risk before transfusion in children with sickle cell disease and not to rely solely on the value of hemoglobin. Indeed, it is important to remember that a negative antibody screen as well as a negative serological crossmatch test do not totally eliminate alloimmunisation or the risk of post-transfusion haemolysis. Sickle cell patients demonstrate multiple immunohaematological features: variant phenotypes (especially in the Rh andMNSsystems), rare blood groups, allo- and auto-immunisation favored because of the inflammatory state. The presence of an alloimmunisation can lead to a significant delay to obtain compatible red blood cell units, a supply difficulty or even a genuine blood transfusion deadlock. About 30 % of the requests for rare blood in France concerns sickle cell patients. Any vaso-occlusive crisis occurring within 3 to 15 days after a transfusion should be suspected to be a delayed haemolytic posttransfusion reaction; whenever necessary, the child should be referred to a reference center and any further transfusions should be avoided. The so-called hyperhaemolysis syndrome, corresponding to a major delayed haemolysis with concomitant destruction of autologous red blood cells, constitutes a major complication of the transfusion and may be potentially fatal. It is essential to educate patients and physicians on the recognition of the clinical signs of delayed haemolytic post-transfusion reactions, in order to rapidly implement measures to limit their immediate effects and avoid their occurrence in case of future transfusion. [ABSTRACT FROM AUTHOR]