Introduction: In human physiology, the spleen is generally neglected, and its role is considered anecdotal. In sickle cell disease, splenic dysfunction is the main cause of life-threatening complic...

Considering the spleen in sickle cell disease

Introduction Sickle cell disease (SCD) is a hereditary red blood cell disorder of public health importance globally with Nigeria the epicenter zone in Africa. There is a paucity of knowledge on how hemoglobin variants, personal characteristics, and environment (socio-demographic triangle) interact to influence SCD propagation. A clinical overview of these epidemiologic parameters may proffer strategies for controlling the SCD disease burden. The objective of this study was to examine the prevalence patterns of SCD, including other associated epidemiologic and hematological (i.e., hemoglobin concentration, ABO blood groups) parameters from laboratory data. Methods this was a retrospective cross-sectional study of 138 newly diagnosed SCD patients in the laboratory unit of the department of haematology using routine alkaline cellulose acetate hemoglobin electrophoresis technique from 2013 to 2014. Demographic and other relevant data were obtained from case notes and laboratory records at the presentation. The agent-host-environment variables were used in the construction of the epidemiological triad chain of transmission. Results a total of 138 (1.63%) newly diagnosed SCD patients aged 7 months to 41 years made up of 39% (0.63% SCD prevalence) adults and 61% (1% SCD prevalence) pediatric age-groups were seen out of 8457 consecutive patients screened within the study period. About 98.55% and 1.45% were homozygous sickle-hemoglobin (SS) and heterozygous sickle-hemoglobin C (SC) variants, respectively. The pediatric department (CHER+CHOP) recorded the highest proportion of SCD (65%). In contrast, the public health department had the least proportion (1%). There was a statistically significant difference in the gender status and the months of SCD diagnosis (p=0.0147). The month of April had the highest proportion of SCD. A majority (66.7%) of the SCD had moderate grade anemia. Conclusion the study revealed a gender disparity in the months of SCD diagnosis. However, there was no statistical difference in the pediatric and adult SCD prevalence patterns.

An overview of sickle cell disease from the socio-demographic triangle - a Nigerian single-institution retrospective study

In Ghana, uncomplicated malaria and sickle cell disease (SCD) is common, hence comorbidity is not farfetched. However, the extent of oxidative stress and the array of clinical manifestations in this comorbidity (presence of both malaria and SCD) has not been fully explored. This study highlights the impact of uncomplicated malaria on SCD. The level of isoprostane, 8-iso-prostaglandin F2α (8-iso-PGF2α) was used to assess oxidative stress while plasma biochemistry and urinalysis was used to assess renal function. Hematological profiling was also done to assess the impact of comorbidity on the hematological cell lines. Of the 411 study participants with malaria, 45 (11%) had SCD. Mean body temperature was significantly higher in comorbidity compared to malaria and SCD cohorts, while a lower parasite density range was obtained in comorbidity compared to malaria cohorts. Furthermore, in comorbidity, the 8-iso-PGF2α oxidative stress biomarker was significantly elevated in all ages, parasite density ranges and gender groups. Comorbidity affected both leukocytic and erythrocytic cell lines with significant eosinophilia and monocytosis coexisting with erythrocytic parameters consistent with severe anemia. Biochemically, while plasma creatinine and bilirubin were significantly elevated in comorbidity, spot urinary creatinine was significantly reduced. Additionally, urine samples in the comorbid state were slightly acidic and hypersthenuric with significant hematuria, proteinuria, and bilirubinemia. Finally, 80% or more malaria-SCD presented with chills, fever, anorexia, headache, joint pains, lethargy, and vomiting. In conclusion, malaria could induce vaso-occlusive crisis in sickle cell disease, therefore, prompt management will alleviate the severity of this comorbidity.

Oxidative stress and associated clinical manifestations in malaria and sickle cell (HbSS) comorbidity

Sickle cell anaemia (SCD) is a life-threatening haematological disorder which is predominant in sub-Saharan Africa and is triggered by a genetic mutation of the β-chain haemoglobin gene resulting in the substitution of glutamic acid with valine. This mutation leads to the production of an abnormal haemoglobin molecule called haemoglobin S (HbS). When deoxygenated, haemoglobin S (HbS) polymerises and results in a sickle-shaped red blood cell which is rigid and has a significantly shortened life span. Various reports have shown a strong link between oxidative stress, inflammation, the immune response, and the pathogenesis of sickle cell disease. The consequence of these processes leads to the development of vasculopathy (disease of the blood vessels) and several other complications. The role of the immune system, particularly the innate immune system, in the pathogenesis of SCD has become increasingly clear in recent years of research; however, little is known about the roles of the adaptive immune system in this disease. This review examines the interaction between the immune system, inflammation, oxidative stress, blood transfusion, and their effects on the pathogenesis of sickle cell anaemia. 

A Review of the Relationship between the Immune Response, Inflammation, Oxidative Stress, and the Pathogenesis of Sickle Cell Anaemia

Haemoglobinopathies and G6PD deficiency are inherited disorders found mostly in malaria-endemic areas among different tribal groups of India. However, epidemiological data specific to Particularly Vulnerable Tribal Groups (PVTGs), important for planning and implementing malaria programmes, is limited. Therefore, the present community-based study aimed to assess the prevalence of haemoglobinopathies and G6PD deficiency among the 13 PVTGs found in the state of Odisha, reporting the maximum malaria cases in the country.This cross-sectional study was conducted from July 2018 to February 2019 in 12 districts, home to all 13 PVTGs, in an estimated sample size of 1461, selected two-stage sampling method. Detection of haemoglobinopathies was done by the variant analyser. Screening of G6PD deficiency was carried out using DPIP method followed by quantification using spectrophotometry. The PCR-RFLP technology was used to determine variant of G6PD deficiency and haplotype analysis of sickle cell, while ARMS-PCR and GAP-PCR was used for detecting the mutation pattern in β-thalassaemia and α-thalassaemia respectively. The diagnosis of malaria was done by Pf-PAN RDT as point of care, followed by nPCR for confirmation and Plasmodium species identification.The prevalence of sickle cell heterozygotes (AS) was 3.4%, sickle cell homozygous (SS) 0.1%, β-thalassaemia heterozygotes 0.3%, HbS/β-thalassaemia compound heterozygote 0.07%, HbS-α-thalassaemia 2.1%, G6PD deficiency 3.2% and malaria 8.1%. Molecular characterization of βS revealed the presence of Arab-Indian haplotype in all HbS cases and IVS 1-5 G → C mutation in all β-thalassaemia cases. In case of α-thal, αα/α-3.7 gene deletion was most frequent (38%), followed by αα/α-4.2 (18%) and α-3.7/α-3.7 (4%). The frequency of G6PD Orissa (131C → G) mutation was found to be 97.9% and G6PD Mediterranean (563C → T) 2.1%. Around 57.4% of G6PD deficient individuals and 16% of the AS were found to be malaria positive.The present study reveals wide spread prevalence of sickle cell anaemia, α-thalassaemia, G6PD deficiency and malaria in the studied population. Moderate to high prevalence of G6PD deficiency and malaria warrants G6PD testing before treating with primaquine (PQ) for radical cure of Plasmodium vivax. Screening and counselling for HbS is required for the PVTGs of Odisha. 

https://malariajournal.biomedcentral.com/counter/pdf/10.1186/s12936-022-04358-5

A community based study on haemoglobinopathies and G6PD deficiency among particularly vulnerable tribal groups in hard-to-reach malaria endemic areas of Odisha, India: implications on malaria control

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. 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. 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.

/pdf/sickle-cell-disease-and-malaria-decreased-exposure-and-4qfgsg6nat.pdf

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

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