Background: The relationship between sickle cell disease and malaria remains controversial and the hypothesis that sickle cell disease protects against malaria is widespread.

Methodology: A descriptive and retrospective study over a two-year period (2014-2016) was conducted in pediatric departments A and B of the National Hospital of Niamey (HNN). The objective is to assess the relative risk between sickle cell disease and the severity of malaria.

Results: Nine hundred and seventy four (974) patients infected with Plasmodium falciparum were included in this study. Thirteen point twenty four percent (129/974) of patients had sickle cell disease, of which 93.8% (121/129) had SS form and 6.2% (8/129) SC form. Seventy-nine point eight percent (103/129) of sickle cell patients had severe malaria (RR = 0.9, p = 0.17). Ninety six point one percent (99/103) of patients with severe malaria have SS hemoglobin versus 3.8% (4/103) who have SC hemoglobin  (RR = 0.6, p = 0.05). Eleven point forty three percent (4/35) of sickle cell patients died of malaria (RR = 0.1, p = 0.4). Seventy-five percent (3/4) of the deceased sickle cell have SS hemoglobin versus 25% (1/4) who have SC hemoglobin (RR = 5, p = 0.2).

Conclusion: Heterozygous sickle cell patients have less severe malaria than homozygotes. Malaria is more severe and more lethal in homozygous sickle cell patients. A strategy for the prevention of sickle cell malaria should be developed during periods of high transmission.

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