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

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, an...

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Autores principales: Mwaiswelo, Richard O., Mawala, William, Iversen, Per O., de Montalembert, Mariane, Luzzatto, Lucio, Makani, Julie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7183641/
https://www.ncbi.nlm.nih.gov/pubmed/32334583
http://dx.doi.org/10.1186/s12936-020-03212-w
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author Mwaiswelo, Richard O.
Mawala, William
Iversen, Per O.
de Montalembert, Mariane
Luzzatto, Lucio
Makani, Julie
author_facet Mwaiswelo, Richard O.
Mawala, William
Iversen, Per O.
de Montalembert, Mariane
Luzzatto, Lucio
Makani, Julie
author_sort Mwaiswelo, Richard O.
collection PubMed
description 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 pubmed-71836412020-04-29 Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum Mwaiswelo, Richard O. Mawala, William Iversen, Per O. de Montalembert, Mariane Luzzatto, Lucio Makani, Julie Malar J Case Report 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. BioMed Central 2020-04-25 /pmc/articles/PMC7183641/ /pubmed/32334583 http://dx.doi.org/10.1186/s12936-020-03212-w Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Mwaiswelo, Richard O.
Mawala, William
Iversen, Per O.
de Montalembert, Mariane
Luzzatto, Lucio
Makani, Julie
Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
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 Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7183641/
https://www.ncbi.nlm.nih.gov/pubmed/32334583
http://dx.doi.org/10.1186/s12936-020-03212-w
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