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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.

Introduction Il existe un risque transfusionnel de transmission du VIH, des hepatites B et C et d’allo-immunisation anti-erythrocytaire pour toute population transfusee comme les drepanocytaires. Notre etude avait pour objectif de determiner la seroprevalence du VIH, VHB et VHC, d’evaluer l’allo-immunisation anti-erythrocytaire chez les sujets drepanocytaires majeurs suivis au service d’hematologie clinique du CNTS. Materiels et methodes Cette etude prospective a ete menee sur une periode de 6 mois. Chaque patient a beneficie du depistage de l’Ag-HBs, l’Ac Anti-VHC, Ac/Ag VIH et de la recherche des agglutinines irregulieres (RAI). Resultats Notre population d’etude est composee de 250 patients drepanocytaires majeurs dont 107 soit 42,8 % etaient de sexe masculin et 143 soit 57,2 % de sexe feminin. Les patients etaient âges de 2 a 59 ans avec un âge moyen de 24,84 ± 9 ans. Parmi ces sujets drepanocytaires 150 ayant recu au moins une transfusion avaient beneficie de la RAI. Les seroprevalences etaient de 2,4 % pour le VHB et de 1,2 % pour le VHC. Tous les patients etaient negatifs pour le VIH. La frequence de l’immunisation etait estimee a 22,66 % avec une predominance des anti-Kell et anti-Rhesus respectivement de 23,67 % et 34,19 %. Le nombre d’unites de sang transfuse est significativement associe a la positivite de la RAI ( p = 0,03). Conclusion La prevalence VIH, VHB et VHC chez les drepanocytaires transfuses etait quasi similaire a celle retrouvee chez les drepanocytaires non transfuses. Par contre, la frequence de l’immunisation anti-erythrocytaire etait elevee dans notre etude.

Background: Sickle cell trait carriers has long considered asymptomatic. This affirmation is now challenged because many patients complain of osteoarticular pain and several organic degenerative complications in particular; renal, eye and sudden death have been described. The objective of this study was to evaluate the morbidity of sickle cell trait and identify risk factors associated.Methods: This is a prospective study with duration of 16 months including 50 patients with sickle cell trait received regular visits (every 6 months) for painful events. Biological assessment was carried out systematically to eliminate rheumatic disease (CRP, ASLO, latex Waler Rose) or metabolic disorders (serum calcium, serum magnesium, and serum uric acid). A correlation between clinical and laboratory data was performed to study the relationship between morbidity observed and biological abnormalities.Results: Mean age of patients was 32 years (12-59) and mean age at diagnosis was 24 years (12-55 years). Sex ratio M/F was 0.16. Clinical symptoms were osteoarticular pain (88%), headache (86%), abdominal pain (76%), muscle cramps (70%), dizziness (56%), biliary lithiasis (6%), femoral head osteonecrosis (2%) and gross haematuria (2%). Seventeen patients (34%) had abnormal metabolic or rheumatic analysis. No risk factor associated with morbidity of patients was identified.Conclusions: This work has allowed us to find that the symptoms presented by sickle cell trait patients are dominated by painful events. This morbidity associated with porting sickle cell trait was not secondary to inflammatory or metabolic disorders or physical activity.