Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum

Abstract Background Patients with sickle cell disease (SCD), an inherited haemoglobinopathy, have increased risk of malaria, at least in part due to impaired splenic function. Infection with Plasmodium falciparum in SCD patients can trigger painful vaso-occlusive crisis, increase the severity of ana...

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Main Authors: Richard O. Mwaiswelo, William Mawala, Per O. Iversen, Mariane de Montalembert, Lucio Luzzatto, Julie Makani
Format: Article
Language:English
Published: BMC 2020-04-01
Series:Malaria Journal
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12936-020-03212-w
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author Richard O. Mwaiswelo
William Mawala
Per O. Iversen
Mariane de Montalembert
Lucio Luzzatto
Julie Makani
author_facet Richard O. Mwaiswelo
William Mawala
Per O. Iversen
Mariane de Montalembert
Lucio Luzzatto
Julie Makani
author_sort Richard O. Mwaiswelo
collection DOAJ
description Abstract Background Patients with sickle cell disease (SCD), an inherited haemoglobinopathy, have increased risk of malaria, at least in part due to impaired splenic function. Infection with Plasmodium falciparum in SCD patients can trigger painful vaso-occlusive crisis, increase the severity of anaemia, and contribute to early childhood mortality. Case presentation A 17 year-old Tanzanian male with known SCD was admitted to Muhimbili National Hospital, a tertiary referral centre in Dar-es-Salaam, following an attack of malaria. From 2004 to 2007 the patient had lived in USA, and from 2010 to 2016 in France where, on account of hypersplenism and episodes of splenic sequestrations, in 2014 the spleen was removed. After appropriate clinical and laboratory assessment the patient was re-started on hydroxyurea; and anti-malarial-prophylaxis with proguanil was instituted. The patient has remained well and malaria-free for the following 15 months. Conclusion SCD patients are highly vulnerable to malaria infection, and impaired splenic function is a feature of SCD patients, even in those who still anatomically have a spleen. This patient had a surgical splenectomy and, in addition, had probably lost some of the acquired malaria-immunity by having lived for several years in malaria-free areas. This patient is a compelling reminder that long-term anti-malarial prophylaxis should be offered to all patients with SCD who live in malaria-endemic areas.
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spelling doaj.art-55d9bb9b30b645a0812fdbeed80755122022-12-21T19:02:01ZengBMCMalaria Journal1475-28752020-04-011911510.1186/s12936-020-03212-wSickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparumRichard O. Mwaiswelo0William Mawala1Per O. Iversen2Mariane de Montalembert3Lucio Luzzatto4Julie Makani5Department of Microbiology, Immunology and Parasitology, Hubert Kairuki Memorial UniversityDepartment of Hematology and Blood Transfusion, Muhimbili University of Health and Allied SciencesDepartment of Hematology and Blood Transfusion, Muhimbili University of Health and Allied SciencesDepartment of General Pediatrics and Pediatric Infectious Diseases, Necker-Enfants malades Hospital, Assistance Publique-Hôpitaux de Paris, Université de ParisDepartment of Hematology and Blood Transfusion, Muhimbili University of Health and Allied SciencesDepartment of Hematology and Blood Transfusion, Muhimbili University of Health and Allied SciencesAbstract Background Patients with sickle cell disease (SCD), an inherited haemoglobinopathy, have increased risk of malaria, at least in part due to impaired splenic function. Infection with Plasmodium falciparum in SCD patients can trigger painful vaso-occlusive crisis, increase the severity of anaemia, and contribute to early childhood mortality. Case presentation A 17 year-old Tanzanian male with known SCD was admitted to Muhimbili National Hospital, a tertiary referral centre in Dar-es-Salaam, following an attack of malaria. From 2004 to 2007 the patient had lived in USA, and from 2010 to 2016 in France where, on account of hypersplenism and episodes of splenic sequestrations, in 2014 the spleen was removed. After appropriate clinical and laboratory assessment the patient was re-started on hydroxyurea; and anti-malarial-prophylaxis with proguanil was instituted. The patient has remained well and malaria-free for the following 15 months. Conclusion SCD patients are highly vulnerable to malaria infection, and impaired splenic function is a feature of SCD patients, even in those who still anatomically have a spleen. This patient had a surgical splenectomy and, in addition, had probably lost some of the acquired malaria-immunity by having lived for several years in malaria-free areas. This patient is a compelling reminder that long-term anti-malarial prophylaxis should be offered to all patients with SCD who live in malaria-endemic areas.http://link.springer.com/article/10.1186/s12936-020-03212-wChemoprophylaxisMalariaSickle cell diseaseSplenic dysfunctionSplenectomy
spellingShingle Richard O. Mwaiswelo
William Mawala
Per O. Iversen
Mariane de Montalembert
Lucio Luzzatto
Julie Makani
Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
Malaria Journal
Chemoprophylaxis
Malaria
Sickle cell disease
Splenic dysfunction
Splenectomy
title Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
title_full Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
title_fullStr Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
title_full_unstemmed Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
title_short Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
title_sort sickle cell disease and malaria decreased exposure and asplenia can modulate the risk from plasmodium falciparum
topic Chemoprophylaxis
Malaria
Sickle cell disease
Splenic dysfunction
Splenectomy
url http://link.springer.com/article/10.1186/s12936-020-03212-w
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