Abstract

Sickle cell disease (SCD) is the most common genetic disease in sub-Saharan Africa. The signs and symptoms of SCD usually begin in early childhood. Characteristic features of this disorder include anaemia, repeated infections, and periodic episodes of pain. Malaria is one of the infections that can occur in patients with SCD in endemic countries. Many guidelines recommend antimalarial chemoprophylaxis in these patients, although the debate on which drug should be used is still ongoing. Hydroxyurea (HU), which is considered a safe and effective treatment for both children and adults with SCD, seems to affect the incidence and severity of malaria, although these impacts have yet to be fully demonstrated. We report a case of an eight-and-a-half-year-old Sudanese boy with SCD treated with HU admitted for suspected severe malaria who showed a recrudescence after first-line treatment. Although he had undergone splenectomy and thus belonged to a category of patients at high risk for infectious complications, he was not receiving any malaria chemoprophylaxis. This case emphasises the importance of the routine administration of malaria prophylaxis to children with SCD living in endemic areas, even when they are treated with HU, and especially if they are at high risk for infectious complications because they have undergone splenectomy. There is an urgent need for further research to evaluate the most appropriate regimen and its optimal duration.

Highlights

  • We report a representative case of recrudescent suspected severe malaria in a Sudanese patient affected by Sickle cell disease (SCD) who underwent a splenectomy at the age of 7 years

  • Hyposplenism plays a significant role in the reduced response to P. falciparum in SCD patients, and some studies have reported a higher risk of severe malaria and a higher incidence of parasitemia after splenectomy [13,14]

  • Chemoprophylaxis against malaria is recommended in African guidelines for the management of SCD patients living in endemic areas [4,5]

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Summary

Background

Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Hydroxyurea (HU), which is considered a safe and effective treatment for both children and adults with SCD, seems to affect the incidence and severity of malaria, its impacts have yet to be fully demonstrated [6,7,8]. For this reason, some experts consider HU as an appropriate drug for malaria prophylaxis, without the need to add additional drugs [6,7,8]. He had undergone splenectomy and belonged to a high-risk category, he was not receiving any malaria chemoprophylaxis

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