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In the title compound, [Tb(H2L)2(H2O)3].3Cl.4(H2O).(C2H5OH), the Tb3+ is nine-coordinated in a distorted monocapped square antiprismgeometry by four nitrogen atoms, two oxygen atoms from the ligand molecules of the tridentate N,N'-1,5-bis(pyridylmethylidene) carbonohydrazone) (H2L) and three oxygen atoms of coordinating water molecules. The structure of the complex was elucidated by X-ray diffraction analysis. Suitable crystals were grown by slow evaporation of ethanol solution. The compound crystallizes in the triclinic crystal system with a space group of Pī. The asymmetric unit of the compound contains two neutral ligand molecules, oneterbium ion, three coordinated water molecules, five and half uncoordinated water molecules and one uncoordinated ethanol molecule. In the crystal, the complex cations are linked by hydrogen bonds into layers. These layers, chloride anions and non-coordinating water molecules are connected by O—H···O, O—H···N, O—H···Cl, N—H···O, N—H···Cl and C—H···Cl hydrogen bonds into a three-dimensional structure.

La détermination du Débit de Filtration Glomérulaire (DFG) est importante chez les drépanocytaires du fait qu’ils constituent un groupe de patients chez lesquels des atteintes rénales sont fréquemment décrites notamment l’hyperfiltration glomérulaire. Dès lors, à une époque où les calculateurs en ligne proposent simultanément différentes formules de détermination du DFG, il serait important d’évaluer au sein d’une population noire africaine drépanocytaire l’équivalence entre ces formules qui ont été développées et validées sur des populations caucasiennes et afro-américaines à DFG normal ou diminué. Ainsi cette étude avait pour but d’évaluer l’interchangeabilité des différentes formules de détermination du DFG en les appliquant à des drépanocytaires. Des enfants et adultes sénégalais drépanocytaires homozygotes ont été alors recrutés et leur DFG calculé. La fréquence de l’hyperfiltration glomérulaire et celle de l’insuffisance rénale ont été calculées à partir des résultats obtenus avec les formules de Schwartz et du CKD-EPI. La concordance des différentes formules a été évaluée avec la méthode Bland-Altman. Au total 56 adultes et 62 enfants ont été inclus dans l’étude. L’insuffisance rénale a été notée chez 1,78% des adultes et 9,68% des enfants ; l’hyperfiltration glomérulaire chez 66,10% des adultes et 25,8% des enfants. Par rapport aux formules de référence (CKD-EPI, Schwartz), tous les biais relevés étaient significativement différents de zéro à l’exception de celui de Cockcroftet Gault qui était statistiquement nul. Les limites de concordance étaient toutes inacceptablement larges par rapport aux limites attendues à l’exception de celles du CKD-EPI sans ajustement sur la race. Ainsi, la formule de Schwartz n’était pas interchangeable avec celle du JSCCS chez les enfants, tout comme celle du CKD-EPI ne l’était pas non plus avec celles du JSCCS, de Cockcroft, du MDRD ou du CKD-EPI sans ajustement sur la race chez les adultes drépanocytaires. English title: Determination of glomerular filtration rate in sickle cell disease in Senegal: Schwartz, Cockcroft and Gault, MDRD, CKD-EPI or JSCCS? Determination of Glomerular Filtration Rate (GFR) is important in patients living with sickle cell disease (SCD) because they constitute a group of patients where kidney dysfunction is frequently described, in particular glomerular hyperfiltration. Therefore, at a time when online calculators simultaneously propose different formulas to estimate GFR, it would be important to evaluate in a black African population living with SCD the equivalence between these formulas which have been developed and validated on Caucasian and African American populations with normal or decreased GFR. Thus, the aim of this study was to evaluate interchangeability of different GFR formulas in a group of patients living with SCD. Homozygous Senegalese sickle cell children and adults were then recruited and their GFR computed using Schwartz and JSCCS in children, Cockcroft and Gault, CKD-EPI with and without adjustment for ethnicity, MDRD and JSCCS formulas in adults. The frequency of glomerular hyperfiltration and renal failure was computed based on the results generated using Schwartz and CKD-EPI formulas. The agreement between formulas was assessed with Bland-Altman method. A total of 56 adults and 62 children were included in this study. Renal failure was observed in 1.78% of adults and 9.68% of children; glomerular hyperfiltration in 66.10% of adults and 25.8% of children. Compared with reference formulas (CKD-EPI, Schwartz), all biases found were significantly different from zero except for Cockcroft and Gault formula bias, which was statistically zero. The limits of agreement were all unacceptably wide compared with the expected limits with the exception of CKD-EPI without adjustment for ethnicity. Thus, Schwartz formula would not be interchangeable with JSCCS formula in children, nor was the CKD-EPI formula interchangeable with the JSCCS, Cockcroft and Gault, MDRD or CKD-EPI without adjustment for ethnicity formulas in adults living with sickle cell anemia.

We evaluated the relevance of using the smudge cell percentage in the blood smear as a prognostic marker in CLL.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.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).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.

Acute panmyelosis with myelofibrosis (APMF) is a subtype of acute myeloid leukemia (AML) classified among the category of “AML, not otherwise specified” in the WHO 2016 classification of hematopoietic tumors. It is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and bone marrow fibrosis in the absence of splenomegaly or fibrosis related morphological changes in the red blood cells. The difficulty of diagnosis and management explains why APMF is rarely reported in Africa. We report here the case of a 30-year-old man who presented with dizziness, palpitations and dyspnea. Diagnosis of APMF was retained on bone marrow histology and immunohistochemistry which showed bone marrow fibrosis and high cellularity with majority of myeloid blast cells. The patient was treated by low dose cytarabine monotherapy 30 mg/m2 per week. At 3 months of treatment, the patient was transfusion-independent, with normalization of hemoglobin and platelets counts. However, the death occurred after 8 months. This case highlights the diagnosis specificity and management of AMPF, knowing the number of potential differential diagnoses and difficulties of its therapeutic management.

Carbonohydrazide was used for synthetizing a new dissymmetrical bis -substituted Schiff base 1-(2'-hydroxybenzylidene)-5-(1'-pyridylethylidene)carbonohydrazone (2). A mono substituted compound (1-(pyridin-2-yl)ethylidene)carbonohydrazide (1) was firstly prepared by condensation reaction of carbonohydrazide and 2-acetylpyridine in 1:1 ratio. Secondly, compound 2 was obtained by condensation reaction of compound 1 and salicylaldehyde in 1:1 ratio. The prepared compounds were characterized by elemental analysis, infrared and 1 H and 13 C NMR spectroscopy techniques, and the structure of compound 2 was determined by single-crystal X-ray diffraction study. The compound 2 (C 15 H 15 N 5 O 2 ) crystallises in the monoclinic space group P 2 1 / c with the following unit cell parameters: a = 8.3683(3) A, b = 13.9986(4) A, c = 12.1610(4) A, β = 97.512(3)°, V = 1412.37(8) A 3 , Z = 4, T = 100(2) K, μ(MoKα) = 0.098 mm -1 , Dcalc = 1.398 g/cm 3 , 6057 reflections measured (5.708° ≤ 2Θ ≤ 54.962°), 6057 unique (R sigma = 0.0395) which were used in all calculations. The final R 1 was 0.0474 (I > 2σ(I)) and wR 2 was 0.1971 (all data). The oxygen atom O1 and the azomethine nitrogen atom N5 adopt cis -configuration relative to the C8-N4 bond, while O1 adopts trans -configuration with the azomethine nitrogen atom N2 relative to C8-N3 bond. The crystal packing of compound 2 is stabilized by intramolecular O(phenol)–H···N(carbohydrazide) and intermolecular N (carbohydrazide)–H···O (carbo-hydrazide) hydrogen bonds which form layers parallel to [010] axis. Additional C–H···O hydrogen bond consolidate the structure. The carbonohydrazide moiety C=N–N–C(O)–N–N=C fragment and the phenyl ring are almost coplanar; with an angle of 1.73(1)° between their means plans. The dihedral angle between the mean planes of the phenyl and the pyridine rings is 22.267(2)°.

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.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.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).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.

A symmetrical dicarbonohydrazide was used to synthesize a centrosymmetric tetra­nuclear zinc(II) complex in which two of the zinc cations are penta­coordinated and the other two are hexa­coordinated., A tetra­nuclear ZnII complex, [Zn4(C13H11N6O)2Cl6(H2O)2] or {[Zn2(HL)(H2O)(Cl2)](μCl)2[Zn2(HL)(H2O)(Cl)]}2, was synthesized by mixing an equimolar amount of a methanol solution containing ZnCl2 and a methanol solution containing the ligand H2 L [1,5-bis­(pyridin-2-yl­methyl­ene)carbono­hydrazide]. In the tetra­nuclear complex, each of the two ligand mol­ecules forms a dinuclear unit that is connected to another dinuclear unit by two bridging chloride anions. In each dinuclear unit, one ZnII cation is penta­coordinated in a N2OCl2 in a distorted square-pyramidal geometry, while the other ZnII cation is hexa­coordinated in a N3OCl2 environment with a distorted octa­hedral geometry. The basal plane around the penta­coordinated ZnII cation is formed by one chloride anion, one oxygen atom, one imino nitro­gen atom and one pyridine nitro­gen atom with the apical position occupied by a chloride anion. The basal plane of the hexa­coordinated ZnII cation is formed by one chloride anion, one hydrazinyl nitro­gen atom, one imino nitro­gen atom and one pyridine nitro­gen atom with the apical positions occupied by a water oxygen atom and a bridged chloro anion from another dinuclear unit, leading to a tetra­nuclear complex. A series of intra­molecular C—H⋯Cl hydrogen bonds is observed in each tetra­nuclear unit. In the crystal, the tetra­nuclear units are connected by inter­molecular C—H⋯Cl, C—H⋯O and N—H⋯O hydrogen bonds, forming a planar two-dimensional structure in the ac plane.

A new Mn(II) complex prepared by the reaction of 1,5-bis(1-(pyridin-2-yl)ethylidene)carbonohydrazide (H2L) with Mn(II) ion is reported in this paper. The H2L ligand is structurally characterized by elemental analysis, NMR and infrared spectroscopies. The mononuclear complex [Mn(H2L)(Cl2)(H2O)] (1), is characterized by infrared spectroscopy, elemental analysis, conductance, magnetic room temperature measurement and single X-ray diffraction. The compound crystallizes in the triclinic system in the space group P-1 with the unit cell parameters a = 8.5453 (13) A, b = 9.4231 (10) A, c = 12.2273 (17) A, α = 78.168 (10)°, β = 81.361 (13)° and γ = 83.187 (11)°, Z = 2, R1 = 0.084 and wR2 = 0.257 (I > 2σ(I)). H2L acts as a tridentate ligand which coordinated metal atom through one pyridine nitrogen atom, one hydrazone nitrogen atom and one hydrazone oxygen atom. The Mn2+ ion has a distorted octahedral environment with three atoms from the ligand, one water oxygen atom and two chloride atoms. Numerous hydrogen bonds form layers propagating in bc plane and ensure the cohesion of crystalline structure in three-dimensional network.

In Africa, acute rheumatic fever is endemic. Cardiac involvement is one of the most common complications in the form of valvular heart disease representing all damages to the heart valves. It is in this perspective that we realized this study whose general objective was to evaluate the results of mitral repair surgery in children in Senegal and the specific objective was to state the indications for mitral repair surgery and assess the results in terms of morbidity and mortality. This is a retrospective and analytical monocentric study, in the thoracic and cardiovascular surgery department of FANN National University Hospital Center in Dakar. It took place over a period of 30 months. All the patients who underwent mitral surgery, aged less than 18 years were included. The total number of patients was 63, including 39 girls and 24 boys, a sex ratio of 0.62. The average age at the time of the surgery was 12 years old [5 - 17]. The functional symptomatology was dominated by the dyspnea found in all the patients. Cardiac ultrasound was diagnosed with mitral regurgitation in all patients. For all surgical procedures, the approach was a vertical midline sternotomy. The mitral valve was approached by left atriotomy in 40 patients (63.5%) and by transseptal way in 23 patients. All patients had undergone mitral valve repair associated or not with either aortic valve repair in 9 patients (14.2%), aortic valve replacement in 3 patients (4.8%), or one tricuspid valve repair in 31 patients (49%). The average duration of intubation was 6 hours [2 - 52]. The average length of stay in intensive care was 2 days. Postoperative complications have been observed. Surgery was performed in 10 patients with 3 mitral valve replacements, 2 aortic valve replacements, a double mitral and an aortic valve replacement associated with a tricuspid repair and in 4 cases a perfection of their mitral repair. Early and late surgical mortality was zero. The average follow-up time for our patients was 9 months [1 - 26]. During their follow-up, the evolution was favorable in 89% of patients who no longer had any functional symptoms.

The ligand 1-(1-(pyridin-2-yl)ethylidene)carbonohydrazide (H4L1) and 1-(pyridin-2-ylmethylene)carbonohydrazide (H4L2) were prepared by reaction of carbonohydrazide with 2-acetylpyridine or pyridine carbaldehyde respectively in a reflux methanol solution. The complexes are prepared by reaction of the ligand with the appropriate metal salt. These complexes are well characterized by elemental analysis, IR and UV spectroscopies and their structure were determined by single X-ray diffraction technic. In the crystal of the dinuclear complex [Mn2(H4L1)2(H2O)4]·Cl4, 1) each Mn(II) center is seven coordinated by two nitrogen atom and one carbonyl atom of the one ligand and one nitrogen atom and one carbonyl oxygen atom of another ligand molecule. The coordination sphere is completed by two water molecules. Each of the carbonyl oxygen atom acts as bridge between the two Mn(II) centers. In the mononuclear complex [Co(H4L2)(NO3)(H2O)2]·(NO3); 2) the Co(II) center is heptacoordinated. The ligand acts in tridentate fashion through two nitrogen atoms and one carbonyl oxygen atom. Two water molecules lie in the apical positions. One nitrate group acts in bidentate manner while the other nitrate group remains uncoordinated. In both complexes the coordination polyhedral are best described as a pentagonal bipyramid. The molecules are linked together in each case by multiple hydrogen bond interaction resulting in a three-dimensional network. The crystallographic data has been deposited in Cambridge Crystallographic Data Centre [CCDC No. 1944387 (complex 1) and 1944386 (complex 2)].

Hemophilia A carriers have an abnormal X chromosome with a molecular abnormality of FVIII gene. These carriers, long considered to be free of bleeding risk, could have the same symptoms as mild hemophiliacs. This study aim to assess bleeding risk of hemophilia A carriers monitored at the Clinical Hematology Department of Dakar. This is a prospective study of a period of 6 months including 22 hemophilia A carriers aged between 8 and 48 years. Hemophilia carriers were recruited using the genealogical tree of hemophiliacs followed in the service. Their diagnosis was carried out by long range PCR and Sanger sequencing method searching the molecular abnormality responsible for hemophilia in their family. Bleeding risk was determined using a questionnaire consisting of different bleeding symptoms quoted from -1 to 4 according to the severity. Total of different values allow to determine the bleeding score which was pathological if it was greater than or equal to 1. Medium age was 22.5 years (8-48) (SD = 9.28). Four hemophilia A carriers (18.1%) presented bleeding symptoms and had a bleeding score at least 1 (P = 0.02). Menorrhagia was predominant (13.6%) followed by epistaxis (9%), gingivorrhagia (9%), and prolonged bleeding after tooth extraction (9%). Factor VIII level was lower in hemophilia carriers who presented bleeding (42 ± 8.61 UI/l) versus hemophilia carriers without bleeding (100 ± 50.95 UI/l) (P = 0.001). There was no significant correlation between bleeding occurrence and age (P = 0.81), activated patial thromboplastin time value (P = 0.97) and FVIII/Von Willebrand Factor ratio (P = 0.12). One in five hemophilia carriers presented bleeding and the questionnaire was effective to identify hemophilia carriers who had a risk of bleeding.

The aim of this study was to evaluate the maternal and fetal complications in pregnant patients with sickle cell disease (SCD) and find risk factors of stillbirth.We conducted a prospective study in pregnant women with SCD. Demographic characteristics, maternal and fetal morbi-mortality, and outcome of pregnancies were described. Risk factors of fetal loss were evaluated by comparing the parameters of the pregnancies that led to a live birth with those interrupted.We included 70 pregnancies in 58 women with SCD. The average age was 29.3 years. The average gestational age at the start of follow-up was 13 weeks. The occurrence of acute complications was significantly higher during pregnancy compared to the year before (p 0.05). Maternal mortality was 0%. Live birth rate was 80%. Fetal loss rate was 3.9 times higher in previous pregnancies that had not been monitored in hematology (71.8 versus 18.6%). Stillbirth was associated with nulliparity, high leukocytes or platelet counts (p 0.05).Pregnancy in SCD was associated with a high maternal morbidity and stillbirth. Nulliparity, high leucokocytes or platelet count were identified as risk factors of fetal loss.

Introduction. The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. Patients and Method. Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. Results. We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/β0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was −60%. Neither alloimmunization nor viral seroconversion was observed. Conclusion. This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

Chronic myeloid leukemia (CML) is an orphan disease in Africa because of the inaccessibility to specific treatment and the high cost of diagnosis and monitoring patients. The aim of this study was to report CML treatment response in a developing country in the tyrosine kinase inhibitor era. We conducted a longitudinal study of our cohort of CML patients. Socio-demographic, diagnosis, therapeutic, and treatment response parameters were studied. Sokal score, disease phase at diagnosis, delay from diagnosis to treatment, and treatment response were analyzed for their impact on survival. Fifty-five patients with a diagnosis of CML and who received treatment with imatinib for a minimum of 3 months were included in this study. Median follow-up was 170 patient-years. The sex ratio (M/F) was 1.62 and median age at diagnosis was 42 years. At diagnosis, 85.5 % of the patients were in chronic phase (CP), 12.7 % in accelerated phase (AP), and 1.8 % in blast crisis (BC). Sokal risk score distribution was as follows: low risk 29.8 %, intermediate risk 38.3 %, and high risk 31.9 %. Median time from first symptoms to first medical visit was 6.2 months and median time from first medical visit to cytogenetic and or molecular confirmation was 12.4 months. Mean delay time from first medical visit to imatinib initiation was 12.5 months (95 % CI 6.3-18.7). The complete hematologic response (CHR) at 3 months, the major cytogenetic response (MCR) at 12 months, and the major molecular response (MMR) at 24 months were respectively 82.4, 75, and 25 %. The 2-year overall survival rate was 81 %. Advanced phase at the diagnosis, discontinuation of imatinib therapy over 15 % of the time, lack of CHR at 3 months, lack of MCR at 12 months, and progression of the disease during imatinib therapy were associated with a risk of death (p ≤ 0.05). Our data confirm the improved prognosis of CML treated with imatinib in the setting of a developing country. However, response rates are lower than in developed countries, and additional efforts should be made to facilitate early diagnosis and improve access to TKI, treatment compliance, and regular molecular monitoring of patients.

Resume Objectif Ce travail avait pour objectif d’evaluer l’efficacite de la selection medicale a exclure les donneurs de sang en fenetre serologique en comparant la prevalence des agents infectieux (VIH, hepatites B et C, syphilis) des donneurs autorises a faire le don, de celle des donneurs exclus. Materiel et methodes Il s’agissait d’une etude prospective transversale d’une duree de 4 mois effectuee chez les donneurs de sang du CNTS de Dakar. Nous avons effectue un echantillonnage de commodite comparant les donneurs exclus par la selection medicale aux donneurs autorises par rapport aux agents infectieux testes (anticorps anti-VIH, AgHBs, anticorps anti-VHC et syphilis). Resultats Au total, 8219 donneurs de sang etaient inclus. La selection medicale avait autorise 8048 donneurs (97,92 %) et en avait exclu 171 (2,08 %). La prevalence du VIH etait plus elevee chez les donneurs exclus (1,75 % contre 0,05 %) ( p = 0,0003 ; OR = 35,91), de meme que celle de l’AgHBs (12,87 % contre 7,35 %) ( p = 0,006 ; OR = 1,86). Les anticorps anti-VHC etaient trouves chez 0,71 % des donneurs autorises et 0,58 % des donneurs exclus ( p = 0,65 ; OR = 0,82). Seuls les donneurs autorises avaient presente l’infection de la syphilis (0,34 %) ( p = 0,56 ; OR = 0). Conclusion Cette etude a permis de montrer l’efficacite de la selection medicale des donneurs de sang dans sa capacite a exclure les donneurs de sang a haut risque de transmission du VIH et a une moindre mesure du VHB. Cependant, les procedures actuelles de selection medicale ne nous permettent pas d’exclure les donneurs porteurs asymptomatiques du VHC et de la syphilis.

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 Sβ0, 99 SC, and 19 Sβ+) 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.

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

La drepanocytose est caracterisee par une grande variabilite d’expression clinique et biologique qui depend de plusieurs facteurs. L’objectif de ce travail etait d’etudier les perturbations biologiques des patients drepanocytaires homozygotes en phase stationnaire. Il s’agit d’une etude prospective transversale ayant inclus 100 drepanocytaires homozygotes en phase stationnaire d’âge superieur a 15 ans. Chaque patient a beneficie de deux tubes de prelevements pour l’etude des parametres hematologiques et pour les dosages biochimiques. Un questionnaire a ete utilise pour recueillir les variables epidemiologiques, cliniques et biologiques. Ces donnees ont ete saisies et analysees par le logiciel Epi-info 7.2. Une valeur p ≤ 0,05 a ete consideree comme significative. L’âge moyen des patients etait de (28 ± 8,94) ans, les extremes (15-57 ans) et le sex-ratio (H/F) de 0,75. Le taux d’hemoglobine de base etait en moyenne de (8,2 ± 1,4g/dl), les globules blancs de (12352 ± 6.906/ mm3), les plaquettes (439.920 ± 139.000/mm3), la TCMH (31,18pg ± 4,12 pg). Le taux d’hemoglobine S etait en moyenne de (87,79 ± 8,19 %) et l’hemoglobine F (HbF) (9,5 ± 8,3%). Le taux HbF etait associee de facon significative avec le syndrome thoracique aigu et la lithiase biliaire (r=0,56 ; p=0,003). Cette etude a montre que les perturbations biologiques sont frequentes chez les dans la drepanocytaires homozygotes. Leur connaissance servira de base de comparaison lors des crises et pour evaluer l’efficacite du traitement.© 2017 International Formulae Group. All rights reserved.Mots cles: Drepanocytose SS, phase stationnaire, perturbations biochimiques, hematologiquesEnglish Title: Biochemical and hematological profile of homozygous sickle cell patients in stationary phase at the Blood Transfusion National Center of DakarEnglish AbstractSickle cell disease is frequently associated with a high clinical and biological variability. This study aimed to describe biological abnormalities in Senegalese patients with homozygous sickle cell disease. In a cross-sectional study, we included 100 patients aged 15 years with homozygous sickle cell disease. Hematological and biochemical parameters were assessed for each patient. Clinical, epidemiological and biological variables were collected using a questionnaire. Data were analyzed using Epi-info 7.2. The mean age of patients was (28 ± 8.94) years and sex ratio (H/F) was 0, 75. The basal hemoglobin level was (8.2 ± 1.48 g / dl), white blood cells (12352 ± 6.906/ mm3), platelets (439.920 ± 139.000/ mm3), TCMH (31, 18 ± 4.12 pg). The mean Hemoglobin S was (87.79 ± 8.19%) and hemoglobin F (HbF) (9.5 ± 8.3%). HbF was associated with acute chest syndrome and biliary lithiasis (r=0, 56; p=0,003). This study showed that biological abnormalities in homozygous sickle cell patients in the stationary phase are frequent. The base line characteristics will help for comparison during next crises and for assess effectiveness of treatment.© 2017 International Formulae Group. All rights reserved.Keywords: Sickle cell disease, stationary phase, biochemical disturbances, hematological

Key Clinical Message RDD (Rosai Dorfman disease) is a rare and benign histiocytic proliferative disorder of unknown etiology. FNAC (Fine-needle aspiration cytology) is a useful and reliable tool for the diagnosis of RDD, and as such, biopsy is avoidable.

Background Circumcision in hemophiliacs is a delicate surgery because of bleeding risks that could be avoided by adequate substitution of coagulation factor. This practice is very challenging in countries where anti hemophilic treatment is inaccessible. The study aimed to evaluate a circumcision protocol in hemophilia A using low quantities of factor concentrates. Methods This prospective study included 26 hemophiliacs A who underwent circumcision in 2014. Medical treatment protocol using low quantity of factor concentrates was drafted by physicians of the Hemophilia Treatment Center and the surgical protocol by experienced surgeons. Assessment criteria were: number of hospitalization days, number of exposure days to factor concentrates, delay to healing and occurrence of bleeding events. Results Mean age was 9.6 years (1–30). Hemophiliacs patients were classified as severe (n = 8), moderate (n = 9) and mild form (n = 9). Mean number of exposure days to factor VIII concentrates was 6.9 days (5–12) in children and 10.75 days (7–16) in adults (p = 0.0049); mean number of hospitalization days was 3.68 days (2–10) in children and 13.5 days (13–15) in adults (p = 0.0000); delay to healing was 26.47 days (20–35) in children and 25.25 days (22–30) in adults (p = 0.697); five haemophiliacs (19.2%) presented bleeding events after the circumcision. The mean amount of FIII concentrates used per patient was 1743 IU (810–2340). Conclusion The study shows treatment protocol using low quantity of factor concentrates is efficient in hemophilia patients who underwent circumcision.