Skip to main content
Top
Published in: Malaria Journal 1/2020

Open Access 01-12-2020 | Plasmodium Falciparum | Case report

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

Authors: Richard O. Mwaiswelo, William Mawala, Per O. Iversen, Mariane de Montalembert, Lucio Luzzatto, Julie Makani

Published in: Malaria Journal | Issue 1/2020

Login to get access

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.
Literature
1.
go back to reference Wastnedge E, Patel S, Goh MY, Rudan I. The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis. J Glob Health. 2018;8:021103.CrossRef Wastnedge E, Patel S, Goh MY, Rudan I. The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis. J Glob Health. 2018;8:021103.CrossRef
2.
go back to reference Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;76:1561–73.CrossRef Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;76:1561–73.CrossRef
3.
go back to reference McAuley CF, Webb C, Makani J, Macharia A, Uyoga S, Opi DH, et al. High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya. Blood. 2010;116:1663–8.CrossRef McAuley CF, Webb C, Makani J, Macharia A, Uyoga S, Opi DH, et al. High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya. Blood. 2010;116:1663–8.CrossRef
4.
go back to reference Makani J, Komba AN, Cox SE, Oruo J, Mwamtemi K, Kitundu J, et al. Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization. Blood. 2010;115:215–20.CrossRef Makani J, Komba AN, Cox SE, Oruo J, Mwamtemi K, Kitundu J, et al. Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization. Blood. 2010;115:215–20.CrossRef
5.
go back to reference Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2013;381(9861):142–51.CrossRef Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2013;381(9861):142–51.CrossRef
6.
go back to reference Wiebe A, Longbottom J, Gleave K, Shearer FM, Sinka ME, Massey NC, et al. Geographical distributions of African malaria vector sibling species and evidence for insecticide resistance. Malar J. 2017;16(1):85.CrossRef Wiebe A, Longbottom J, Gleave K, Shearer FM, Sinka ME, Massey NC, et al. Geographical distributions of African malaria vector sibling species and evidence for insecticide resistance. Malar J. 2017;16(1):85.CrossRef
7.
go back to reference Luzzatto L. Sickle cell anaemia and malaria. Mediterr J Hematol Infect Dis. 2012;4(1):e2012065.CrossRef Luzzatto L. Sickle cell anaemia and malaria. Mediterr J Hematol Infect Dis. 2012;4(1):e2012065.CrossRef
8.
go back to reference Tubman VN, Makani J. Turf wars: exploring splenomegaly in sickle cell disease in malaria-endemic regions. Br J Haematol. 2017;177(6):938–46.CrossRef Tubman VN, Makani J. Turf wars: exploring splenomegaly in sickle cell disease in malaria-endemic regions. Br J Haematol. 2017;177(6):938–46.CrossRef
9.
go back to reference Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165–76.CrossRef Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165–76.CrossRef
10.
go back to reference Chotivanich K, Udomsangpetch R, Mcgready R, Proux S, Newton P, Pukrittayakamee S, et al. Central role of the spleen in malaria parasite clearance. J Infect Dis. 2002;185(10):1538–41.CrossRef Chotivanich K, Udomsangpetch R, Mcgready R, Proux S, Newton P, Pukrittayakamee S, et al. Central role of the spleen in malaria parasite clearance. J Infect Dis. 2002;185(10):1538–41.CrossRef
11.
go back to reference Demar M, Legrand E, Hommel D, Esterre P, Carme B. Plasmodium falciparum malaria in splenectomized patients: two case reports in French Guiana and a literature review. Am J Trop Med Hyg. 2004;71(3):290–3.CrossRef Demar M, Legrand E, Hommel D, Esterre P, Carme B. Plasmodium falciparum malaria in splenectomized patients: two case reports in French Guiana and a literature review. Am J Trop Med Hyg. 2004;71(3):290–3.CrossRef
12.
go back to reference Davidson RN, Wall RA. Prevention and management of infections in patients without a spleen. Clin Microbiol Infect. 2001;7(12):657–60.CrossRef Davidson RN, Wall RA. Prevention and management of infections in patients without a spleen. Clin Microbiol Infect. 2001;7(12):657–60.CrossRef
13.
go back to reference Menendez C, Fleming AF, Alonso PL. Malaria-related anaemia. Parasitol Today. 2000;16(11):469–76.CrossRef Menendez C, Fleming AF, Alonso PL. Malaria-related anaemia. Parasitol Today. 2000;16(11):469–76.CrossRef
14.
go back to reference Frimpong A, Thiam LG, Arko-Boham B, Owusu EDA, Adjei GO. Safety and effectiveness of antimalarial therapy in sickle cell disease: a systematic review and network meta-analysis. BMC Infect Dis. 2018;18(1):650.CrossRef Frimpong A, Thiam LG, Arko-Boham B, Owusu EDA, Adjei GO. Safety and effectiveness of antimalarial therapy in sickle cell disease: a systematic review and network meta-analysis. BMC Infect Dis. 2018;18(1):650.CrossRef
15.
go back to reference Oniyangi O, Omari AA. Malaria chemoprophylaxis in sickle cell disease. Cochrane Database Syst Rev. 2006;2006(4):CD003489.PubMedCentral Oniyangi O, Omari AA. Malaria chemoprophylaxis in sickle cell disease. Cochrane Database Syst Rev. 2006;2006(4):CD003489.PubMedCentral
17.
go back to reference Haldar K, Mohandas N. Malaria, erythrocytic infection, and anemia. Hematology Am Soc Hematol Educ Program. 2009;1:87–93.CrossRef Haldar K, Mohandas N. Malaria, erythrocytic infection, and anemia. Hematology Am Soc Hematol Educ Program. 2009;1:87–93.CrossRef
18.
go back to reference Bunn HF. The triumph of good over evil: protection by the sickle gene against malaria. Blood. 2013;121(1):20–5.CrossRef Bunn HF. The triumph of good over evil: protection by the sickle gene against malaria. Blood. 2013;121(1):20–5.CrossRef
19.
go back to reference Doolan DL, Dobano C, Baird JK. Acquired immunity to malaria. Clin Microbiol Rev. 2009;22(1):13–36.CrossRef Doolan DL, Dobano C, Baird JK. Acquired immunity to malaria. Clin Microbiol Rev. 2009;22(1):13–36.CrossRef
20.
go back to reference Farnert A, Wyys K, Dashti S, Naucler P. Duration of residency in a non-endemic area and risk of severe malaria in African immigrants. Clin Microbiol Infect. 2015;21(5):494–501.CrossRef Farnert A, Wyys K, Dashti S, Naucler P. Duration of residency in a non-endemic area and risk of severe malaria in African immigrants. Clin Microbiol Infect. 2015;21(5):494–501.CrossRef
21.
go back to reference El Hoss S, Brousse V. Considering the spleen in sickle cell disease. Expert Rev Hematol. 2019;12(7):563–73.CrossRef El Hoss S, Brousse V. Considering the spleen in sickle cell disease. Expert Rev Hematol. 2019;12(7):563–73.CrossRef
22.
go back to reference Olaosebikan R, Ernest K, Bojang K, Mokuolu O, Rehman A, Affara M, et al. A randomized trial to compare the safety, tolerability, and effectiveness of 3 antimalarial regimens for the prevention of malaria in Nigerian patients with sickle cell disease. J Infect Dis. 2015;212(4):617–25.CrossRef Olaosebikan R, Ernest K, Bojang K, Mokuolu O, Rehman A, Affara M, et al. A randomized trial to compare the safety, tolerability, and effectiveness of 3 antimalarial regimens for the prevention of malaria in Nigerian patients with sickle cell disease. J Infect Dis. 2015;212(4):617–25.CrossRef
23.
go back to reference Lalloo DG, Hill DR. Preventing malaria in travellers. BMJ. 2008;336(7657):1362–6.CrossRef Lalloo DG, Hill DR. Preventing malaria in travellers. BMJ. 2008;336(7657):1362–6.CrossRef
24.
go back to reference Schwartz E. Prophylaxis of malaria. Mediterr J Hematol Infect Dis. 2012;4(1):e2012045.CrossRef Schwartz E. Prophylaxis of malaria. Mediterr J Hematol Infect Dis. 2012;4(1):e2012045.CrossRef
Metadata
Title
Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum
Authors
Richard O. Mwaiswelo
William Mawala
Per O. Iversen
Mariane de Montalembert
Lucio Luzzatto
Julie Makani
Publication date
01-12-2020
Publisher
BioMed Central
Published in
Malaria Journal / Issue 1/2020
Electronic ISSN: 1475-2875
DOI
https://doi.org/10.1186/s12936-020-03212-w

Other articles of this Issue 1/2020

Malaria Journal 1/2020 Go to the issue
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.