Determinants of the haemoglobin level in patients with sickle cell disease living in sub-Saharan Africa: Major impact of the country of residence and independent effects of leucocyte and platelet counts and haemolysis.

The degree of anaemia in sickle cell disease (SCD) is a well-known contributor to morbidity and mortality. We aimed to explore the factors affecting haemoglobin (Hb) level in African SCD patients, considering haemolysis biomarkers (LDH and bilirubin level, and reticulocyte count), leucocyte and platelet counts and socio-demographic characteristics (gender, age group, country of residence and BMI). The research was part of the CADRE multinational cohort and involved 3699 SCD patients living in Mali, Senegal, Ivory Coast, Democratic Republic of Congo, Gabon and Cameroon: 2936 SS/Sß0, 587 SC and 176 Sß + patients with median Hb level of 8, 11.3 and 11.2 g/dL respectively (p < 0.001). In multivariate analysis conducted in 1394 SS/Sß0 patients, living in Cameroon, female gender, lower BMI, higher haemolysis markers (especially LDH) and higher leucocyte and platelet counts were independently associated with lower Hb level (all p < 0.05). In 497 SC and 156 Sß + patients, female gender (p < 0.001), lower BMI (p < 0.05) and higher platelet counts (p < 0.001) were independently associated with lower Hb level. Anaemia in African SCD patients is not only associated with haemolysis but also with the country of residence, lower BMI and leucocyte or platelet counts which might reflect inflammation related to infectious burden in the region.

A Case of Rosai-Dorfman Disease Successfully Treated by Corticotherapy.

Rosai-Dorfman disease (RDD) is a benign histiocytic proliferation that results in nodal and extranodal involvements. It is a rare disease, with fewer than 1,000 cases reported in the literature, which explains its lack of knowledge by physicians and the lack of codified therapeutic strategies. We report the case of an 8-year-old girl who presented a rapidly progressive cervical lymph node mass; the diagnosis of RDD was made based on histology and immunohistochemistry. The patient was treated with oral corticosteroids at a dose of 1 mg/kg/d with a favorable outcome and no recurrence after one year of follow-up. This observation illustrates the clinical presentation and diagnosis of this rare clinicopathological entity. The prognosis and treatment options are also discussed.

Homozygous Sickle Cell Disease after Age of 40: Follow-Up of a Cohort of 209 Patients in Senegal, West Africa.

Objectives: The aim of this study was to describe the morbidity and mortality of homozygous sickle cell disease after the age of 40. Methods: This was a cohort study of 209 patients followed from 1994 to 2022. All hemoglobin electrophoresis-confirmed SS sickle cell patients over 40 years were included. A descriptive study of epidemiological, diagnostic, therapeutic, and evolutionary data was used to assess morbidity and mortality. Results: Sex ratio (M/F) was 0.6. Median age was 47 (41-75). According to morbidity, 95.1% had less than 3 vaso-occlusive crises/year. Acute anemia was the most frequent complication (52.63%). Chronic complications were noted in 32.5%. At diagnosis, mean hemoglobin was 8.1 g/dl ± 1.9, HbS was 86.5 ± 10, and HbF was 9.4 ± 7.6. Number of patients transfused was 66%. We noted that 8.1% of patients died, 29.2% were lost to follow-up, and 62.7% were still being followed up. The risk factors identified for death were geographical origin, comorbidity, high HbS, low HbF, and thrombocytosis. Conclusion: This study shows that homozygous SCD is increasingly becoming an adult disease and that it can be carried into old age in Africa. Advanced age over 40 is marked by an upsurge in chronic complications, making it essential to set up a screening program and to organize multidisciplinary follow-up.

Clinical outcome and incidence of inhibitor development in severe hemophilia patients receiving low-dose prophylaxis: a 3-year follow-up study in Senegal, West Africa

ABSTRACT Introduction: In Africa, where access to diagnosis and treatment of hemophilia is the lowest in the world, prophylaxis is rarely used in preference to on-demand treatment. There are limited data of prophylaxis treatment from sub-Saharan Africa. The aim of this study was to evaluate clinical outcomes and inhibitor development in people with hemophilia receiving low-dose prophylaxis (LDP) in a sub-Saharan African setting. Methods: We conducted a three-year prospective study. A once or twice weekly prophylaxis regimen of 25 IU/kg of rFVIIIFc or 30 IU/kg of rFIXFc was given to Hemophilia A and B, respectively. We evaluated clinical outcomes and inhibitors occurrence, determined by screening and titration using the Nijmegen technique. Results: A total of 15 patients were included in the LDP regimen. The mean age was 6.3 years (1.5 - 10). A significant reduction was noted in the annualized bleeding rate, from 7.53 to 1.33 (p = 0.0001); the annualized joint bleeding rate passed from 3.6 to 1.4 (p = 0.001) and the proportion of severe bleeding, from 86.1% to 16.7% (p = 0.0001). The Hemophilia Joint Health Score (HJHS) moved from 9.6 to 3.4 (p = 0.0001) and the Functional Independence Score in Hemophilia (FISH) improved from 25.8 to 30.9 (p = 0.0001). School absenteeism decreased from 7.33% to 2.59%. Adherence to prophylaxis was 89.5% versus 60%. Consumption was 580 IU/kg/year versus 1254.6 IU/kg/year before and after prophylaxis, respectively. Incidence of inhibitors was 23% (3 /13 HA). Conclusion: The LDP in Hemophilia improves the clinical outcome without a surplus risk of inhibitor development. Using extended half-life clotting factor concentrates (CFCs) is better for prophylaxis in resource-limited countries, as they allow better compliance in treatment.

Evaluation of knowledge of priapism in sickle cell patients in Senegal.

PURPOSE: Erectile dysfunction is a dreadful complication of priapism especially with delay in diagnosis and management. The lack of awareness of priapism as a vaso-occlusive complication of sickle cell disease (SCD) is more concerning. The objective of this study was to evaluate the burden of priapism in adult Senegalese males adults with sickle cell disease. METHODS: A cross-sectional study was carried out amongst consecutive consenting males aged 18 years and older with SCD presenting to the in Haematology Department of the National Blood Transfusion Centre (Dakar, Senegal). All participants completed a questionnaire detailing knowledge on the definition of priapism, its association with SCD, consequences of untreated priapism and treatment options. RESULTS: A total of 219 participants completed the questionnaire. The mean age of the respondents was 27.1 years with a range of (18-54). Of the respondents, 78.5% (n = 172) did not have any knowledge of the term "priapism". After the term was explained, 38.4% (n = 84) thought that there may be a risk of developing priapism given the diagnosis of sickle cell disease. Among the participants, 41.5% (n = 91) reported having a history of priapism. Among all patients who experienced priapism, 36.3% (n = 33) did not seek medical attention with episodes of priapism. It was found that 48.4% (n = 106) of the participants thought there may be a risk of irreversible complications associated with priapism and a corresponding proportion, 42% (n = 92) thought this risk was time dependent. 36.5% (n = 80) of patients believed priapism could lead to erectile dysfunction. CONCLUSION: Priapism is a common complication of sickle cell disease in Senegalese adults which is not well known by sickle cell patients. The health authorities must undertake efforts to raise awareness of priapism as a complication amongst sickle cell patients.

Red blood cell alloantibodies in paediatric transfusion in sub-Saharan Africa: A new cohort and literature review.

Blood transfusion support predisposes transfused children to the risk of erythrocyte alloimmunization in Sub-Saharan Africa. A cohort of 100 children receiving one to five blood transfusions were recruited for screening and identification of irregular antibodies using gel filtration technique. The mean age was 8 years and the sex-ratio at 1.2. The retrieved pathologies were: major sickle cell anaemia (46%), severe malaria (20%), haemolytic anaemia (4%), severe acute malnutrition (6%), acute gastroenteritis (5%), chronic infectious syndrome (12%) and congenital heart disease (7%). The children presented with haemoglobin levels ≤6 g/dl, and 16% of them presented positive irregular antibodies directed against the Rhesus (30.76%) and Kell (69.24%) blood group systems. A literature review shows that irregular antibody screenings vary from 17% to 30% of transfused paediatric patients in Sub-Saharan Africa. These alloantibodies are in particular directed against the Rhesus, Kell, Duffy, Kidd and MNS blood group and generally found in sickle cell disease and malaria. This study highlights the urgent need of extended red blood cell phenotyping including typing for C/c, E/e, K/k, and Fya/Fyb, and if possible Jka/Jkb, M/N, and S/s for children before transfusion in Sub-Saharan Africa.

Enuresis and overactive bladder in sickle cell patients: a narrative review of the literature.

OBJECTIVE: The aim of this review is to clarify the prevalence, pathophysiology and clinical presentation of enuresis and overactive bladder in sickle cell patients. MATERIALS AND METHODS: This narrative review of the literature was conducted in March 2022 by running a search in PubMed, Embase, Scopus and Cochrane databases without publication date limitation, using the following keywords: enuresis or nocturia or overactive bladder or urinary incontinence or bedwetting and sickle cell. RESULTS: Eight cross-sectional studies were included, six of which had a non-sickle cell control population. The prevalence of enuresis in children and adolescents with sickle cell disease ranged from 20.3 to 49.4%. It decreased with age to 2.9% in adult sickle cell patients. Enuresis in sickle cell patients has been attributed to several causes, including lack of urine concentration with nocturnal polyuria, reduced bladder capacity, nocturnal bladder hyperactivity, sleep and/or respiratory disorders are likely causes of enuresis in sickle cell patients. The prevalence of overactive bladder is three times higher in sickle cell patients than in control groups. The latter is also observed three times more frequently in men who have had prior episodes of priapism. CONCLUSION: Enuresis and overactive bladder are common in sickle cell patients. Several mechanisms have been described to try to explain enuresis in sickle cell patients but overactive bladder seems to play a major role. Studies evaluating the efficacy of certain experimentally validated treatments must be carried out to improve the management of these complications which affect the quality of life of sickle cell patients.

Clinical outcome and incidence of inhibitor development in severe hemophilia patients receiving low-dose prophylaxis: a 3-year follow-up study in Senegal, West Africa.

INTRODUCTION: In Africa, where access to diagnosis and treatment of hemophilia is the lowest in the world, prophylaxis is rarely used in preference to on-demand treatment. There are limited data of prophylaxis treatment from sub-Saharan Africa. The aim of this study was to evaluate clinical outcomes and inhibitor development in people with hemophilia receiving low-dose prophylaxis (LDP) in a sub-Saharan African setting. METHODS: We conducted a three-year prospective study. A once or twice weekly prophylaxis regimen of 25 IU/kg of rFVIIIFc or 30 IU/kg of rFIXFc was given to Hemophilia A and B, respectively. We evaluated clinical outcomes and inhibitors occurrence, determined by screening and titration using the Nijmegen technique. RESULTS: A total of 15 patients were included in the LDP regimen. The mean age was 6.3 years (1.5 - 10). A significant reduction was noted in the annualized bleeding rate, from 7.53 to 1.33 (p = 0.0001); the annualized joint bleeding rate passed from 3.6 to 1.4 (p = 0.001) and the proportion of severe bleeding, from 86.1% to 16.7% (p = 0.0001). The Hemophilia Joint Health Score (HJHS) moved from 9.6 to 3.4 (p = 0.0001) and the Functional Independence Score in Hemophilia (FISH) improved from 25.8 to 30.9 (p = 0.0001). School absenteeism decreased from 7.33% to 2.59%. Adherence to prophylaxis was 89.5% versus 60%. Consumption was 580 IU/kg/year versus 1254.6 IU/kg/year before and after prophylaxis, respectively. Incidence of inhibitors was 23% (3 /13 HA). CONCLUSION: The LDP in Hemophilia improves the clinical outcome without a surplus risk of inhibitor development. Using extended half-life clotting factor concentrates (CFCs) is better for prophylaxis in resource-limited countries, as they allow better compliance in treatment.

Biomarkers of sickle cell nephropathy in Senegal.

Sickle cell anemia (SCA) is caused by a single point variation in the ß-globin gene (HBB): c.20A> T (p.Glu7Val), in homozygous state. SCA is characterized by sickling of red blood cells in small blood vessels which leads to a range of multiorgan complications, including kidney dysfunction. This case-control study aims at identifying sickle cell nephropathy biomarkers in a group of patients living with SCA from Senegal. A total of 163 patients living with SCA and 177 ethnic matched controls were investigated. Biological phenotyping included evaluation of glycemia, glucosuria, albuminuria, proteinuria, tubular proteinuria, serum creatinine, urine creatinine, urine specific gravity and glomerular filtration rate. Descriptive statistics of biomarkers were performed using the χ2 -test, with the significance level set at p<0.05. Patients living with SCA had a median age of 20 years (range 4 to 57) with a female sex frequency of 53.21%. The median age of the control participants was 29 years (range: 4-77) with a female sex frequency of 66.09%. The following proportions of abnormal biological indices were observed in SCA patients versus (vs.) controls, as follows: hyposthenuria: 35.3%vs.5.2% (p<0.001); glomerular hyperfiltration: 47.66%vs.19.75% (p<0.001), renal insufficiency: 5.47%vs.3.82% (p = 0.182); microalbuminuria: 42.38%vs.5.78% (p<0.001); proteinuria: 39.33%vs.4.62% (p<0.001); tubular proteinuria: 40.97%vs.4.73% (p<0.001) and microglucosuria: 22.5%vs.5.1% (p<0.001). This study shows a relatively high proportion of SCA nephropathy among patients living with SCA in Senegal. Microglucosuria, proteinuria, tubular proteinuria, microalbuminuria, hyposthenuria and glomerular hyperfiltration are the most prevalent biomarkers of nephropathy in this group of Senegalese patients with SCA.

Effects of Senegal haplotype (Xmn1-rs7412844), alpha-thalassemia (3.7kb HBA1/HBA2 deletion), NPRL3-rs11248850 and BCL11A-rs4671393 variants on sickle cell nephropathy.

OBJECTIVE: Sickle cell anemia (SCA) can cause substantial kidney dysfunction resulting in sickle cell nephropathy, which may be affected by the presence of modifier genes. This study evaluates the effects of some modifier genes on sickle cell nephropathy. METHODS: Patients living with SCA were recruited. Alpha-thalassemia (3.7kb HBA1/HBA2 deletion) was genotyped using gap PCR multiplex. Senegal haplotype (Xmn1-rs7412844), BCL11A-rs4671393 and NPRL3-rs11248850 were genotyped using Mass Array. The effects of variants on kidney dysfunction were then evaluated using multivariate analysis. RESULTS: The number of patients living with SCA included in this study was 162 with a median age of 20 years [minimum-maximum: 4-57] and a female frequency of 53.21%. Senegal haplotype, BCL11A-rs4671393 variant were protective factors against albuminuria stage A2 with an odds ratio (OR) of 0.22 (95% CI 0.05-0.90) and 0.27 (95% CI 0.08-0.96) respectively. The combination NPRL3-rs11248850 variant - 3.7kb HBA1/HBA2 deletion was a protective factor against albuminuria stage A2 (OR = 0.087, 95% Cl 0.01-0.78) but it was a risk factor for glomerular hyperfiltration (OR = 17.69, 95% CI 1.85-169.31). CONCLUSIONS: All four variants displayed a protective effect against albuminuria stage A2. The combination alpha-thalassemia - NPRL3-rs11248850 variant is a risk factor for glomerular hyperfiltration.

Life-threatening bleeding in patients with hemophilia (PWH): a 10-year cohort study in Dakar, Senegal.

OBJECTIVE: The aim of this study was to assess incidence, risk factors, treatment and outcome of LTB in Senegalese people with hemophilia (PWH). METHODS: We analyzed the characteristics of LTB in a cohort of 274 PWH after 10 years of follow-up. RESULTS: We included 274 patients (241 HA and 33 HB). The mean age was 16.45 years and the median age was 13 years. The mean annual bleeding rate (ABR) was 1.65 (2.83 for severe form, 1.54 for moderate form, and 1.22 for mild form). A replacement therapy with clotting factor concentrates (CFC) was administered to 217 patients (79.2%); 56 patients (20.4%) received low-dose prophylaxis (LDP). Prevalence of inhibitors was 4.7% (13/274). All patients were HIV and HCV antibody negative. We observed 31 cases of LTB in 22 patients with an incidence of 8.03%. Central nervous system (CNS) bleeds were most frequent (6.2%) and accounted for 54.8% of severe bleeding. The delay between the first signs and the emergency visit was 78.9 hours. Inhibitors were positive in one patient among those who presented LTB. These bleeding were treated with CFC in 16 patients, surgical drainage (1 patient) and electrocoagulation during gastroscopy (1 patient). Eleven patients had complete remission and two had sequelae. We reported 0.32 death per 100 person-years. CNS bleeds were the main cause (77.7%). Four patients were secondarily on LDP. We observed a significant correlation between treatment (after 2 hours) and mortality. CONCLUSION: LTB is a serious and lethal complication in PWH in absence of early management. A good awareness of patients and their family would further reduce this incidence, especially in resources-limited countries.

Transfusion Practice, Post-Transfusion Complications and Risk Factors in Sickle Cell Disease in Senegal, West Africa.

CONTEXT AND OBJECTIVES: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD) but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization, and iron overload secondary to BT in SCD patients. MATERIALS AND METHODS: This case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload), and risk factors of these complications (socio-demographic, clinical, biological). RESULTS: Median age was 28.5 years (5 - 59). The sex ratio was 0.86. Homozygous SCD was the most common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBCSs) were administered to 93.46%. The median RBCs received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBCs transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission. CONCLUSION: BT therapy is still a risk for SCD polytransfused patients despite advances in blood safety. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.

Smudge cells percentage on blood smear is a reliable prognostic marker in chronic lymphocytic leukemia

Abstract Objective We evaluated the relevance of using the smudge cell percentage in the blood smear as a prognostic marker in CLL. Methods In this prospective study, 42 untreated Senegalese patients with CLL were enrolled. The diagnosis was established, based on the peripheral blood count and flow cytometry using the Matutes score. Cytogenetic aberrations, assessed by fluorescence in situ hybridization (FISH), were available for 30 patients, while the immunoglobulin heavy chain genes (IGVH) mutation status was performed by next-generation sequencing (NGS) in 24 patients. The SC percentage was determined in the blood smear, as previously described. Statistical analyses were executed using the GraphPad Prism 8. Results The mean age was 63 years (48 - 85) and the male: female sex ratio was 4.66. A low SC (< 30%) percentage was correlated with Binet stage B/C (p= 0.0009), CD38 expression (p= 0.039), unmutated IGVH status (p= 0.0009) and presence of cytogenetic abnormalities (for del 13q, p= 0.0012, while for other cytogenetic aberrations, p= 0.016). An inverse correlation was found between the SC percentage and the absolute lymphocyte count (r= -0.51) and patients with higher percentage of SCs had a prolonged survival. However, there was no correlation between the SC percentage and age (p= 0.41) or gender (median, 19% for males vs. 20% for females; p= 0.76). Conclusion When less than 30%, the SC was associated with a poor prognosis in CLL. Easy and affordable, the percentage of SCs in a blood smear could be a reliable prognostic marker, accessible to all CLL patients, mainly those in developing countries.

Smudge cells percentage on blood smear is a reliable prognostic marker in chronic lymphocytic leukemia.

OBJECTIVE: We evaluated the relevance of using the smudge cell percentage in the blood smear as a prognostic marker in CLL. METHODS: In this prospective study, 42 untreated Senegalese patients with CLL were enrolled. The diagnosis was established, based on the peripheral blood count and flow cytometry using the Matutes score. Cytogenetic aberrations, assessed by fluorescence in situ hybridization (FISH), were available for 30 patients, while the immunoglobulin heavy chain genes (IGVH) mutation status was performed by next-generation sequencing (NGS) in 24 patients. The SC percentage was determined in the blood smear, as previously described. Statistical analyses were executed using the GraphPad Prism 8. RESULTS: The mean age was 63 years (48 - 85) and the male: female sex ratio was 4.66. A low SC (< 30%) percentage was correlated with Binet stage B/C (p = 0.0009), CD38 expression (p = 0.039), unmutated IGVH status (p = 0.0009) and presence of cytogenetic abnormalities (for del 13q, p = 0.0012, while for other cytogenetic aberrations, p = 0.016). An inverse correlation was found between the SC percentage and the absolute lymphocyte count (r = -0.51) and patients with higher percentage of SCs had a prolonged survival. However, there was no correlation between the SC percentage and age (p = 0.41) or gender (median, 19% for males vs. 20% for females; p = 0.76). CONCLUSION: When less than 30%, the SC was associated with a poor prognosis in CLL. Easy and affordable, the percentage of SCs in a blood smear could be a reliable prognostic marker, accessible to all CLL patients, mainly those in developing countries.

Homozygous sickle cell disease related mortality in Senegal (2011-2020).

Homozygous sickle cell disease (HSCD) is characterized by multiorgan morbidity and an increased risk of early death. We aim to describe the mortality rate, causes, and risk factors of death in HSCD between 2011 and 2020. We conducted a retrospective study with a duration of 10 years in the cohort of 2348 HSCD patients. The mortality rate was determined by reporting the number of deaths to the total number of patients followed in the year. Sociodemographic, clinical, biological data and causes of death were studied. Death risk factors were determined by a bivariate analysis comparing deceased and living HSCD patients. The mean age of death was 26 years (3-52). The sex ratio was 1.2. The mortality rate was 2.76%. The death rate was high in 2011 (3.2%) and low in 2020 (0.17%). We observed a significant reduction of mortality of 94.6%. Most of the common causes of death were acute anemia (40%), acute chest syndrome (24.6%), and infections (20%). Risk factors of death were age, vaso-occlusive crises ≥3, acute chest syndrome, blood transfusion, and chronic complications. Mortality among HSCD has significantly decreased over the past 10 years in Senegal, and the main causes of death were acute anemia, acute chest syndrome, and infections.

Subclinical Cardiac Dysfunction Is Associated With Extracardiac Organ Damages.

Background: Several studies conducted in America or Europe have described major cardiac remodeling and diastolic dysfunction in patients with sickle cell disease (SCD). We aimed at assessing cardiac involvement in SCD in sub-Saharan Africa where SCD is the most prevalent. Methods: In Cameroon, Mali and Senegal, SCD patients and healthy controls of the CADRE study underwent transthoracic echocardiography if aged ≥10 years. The comparison of clinical and echocardiographic features between patients and controls, and the associations between echocardiographic features and the vascular complications of SCD were assessed. Results: 612 SCD patients (483 SS or Sß0, 99 SC, and 19 Sß+) and 149 controls were included. The prevalence of dyspnea and congestive heart failure was low and did not differ significantly between patients and controls. While left ventricular ejection fraction did not differ between controls and patients, left and right cardiac chambers were homogeneously more dilated and hypertrophic in patients compared to controls and systemic vascular resistances were lower (p < 0.001 for all comparisons). Three hundred and forty nine SCD patients had extra-cardiac organ damages (stroke, leg ulcer, priapism, microalbuminuria or osteonecrosis). Increased left ventricular mass index, cardiac dilatation, cardiac output, and decreased systemic vascular resistances were associated with a history of at least one SCD-related organ damage after adjustment for confounders. Conclusions: Cardiac dilatation, cardiac output, left ventricular hypertrophy, and systemic vascular resistance are associated with extracardiac SCD complications in patients from sub-Saharan Africa despite a low prevalence of clinical heart failure. The prognostic value of cardiac subclinical involvement in SCD patients deserves further studies.

Pulse wave velocity is lower in trained than in untrained sickle cell trait carriers.

BACKGROUND: Sickle cell trait (SCT) is a benign condition of sickle cell disease. Nevertheless, previous reports showed that SCT carriers have increased blood viscosity and decreased vascular reactivity compared to non-SCT carrier. The benefit of regular exercise on vascular function has been well documented in the general population but no study focused on the SCT population. PURPOSE: The aim of our study was to compare arterial stiffness and blood viscosity between trained and untrained SCT carriers, as well as a group of untrained non-SCT. METHODS: Arterial stiffness (finger-toe pulse wave velocity) and blood viscosity were evaluated in untrained non-SCT carriers (n = 10), untrained SCT carriers (n = 23) and trained SCT carriers (n = 17) who reported at least 10 hours of physical exercise per week. RESULTS: Untrained SCT carriers had higher pulse wave velocity (p = 0.032) and blood viscosity (p < 0.001) than their trained counterparts. In addition, untrained SCT carriers had higher blood viscosity (p < 0.001) than the untrained non-SCT group. A positive association was noted between blood viscosity and pulse wave velocity in the whole study population. CONCLUSION: Our study suggests that regular exercise may be beneficial for the vascular function of SCT carriers.