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.

Bleeding risk assessment in hemophilia A carriers from Dakar, Senegal.

: 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 ±â€Š8.61 UI/l) versus hemophilia carriers without bleeding (100 ±â€Š50.95 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.

Circumcision in hemophilia using low quantity of factor concentrates: experience from Dakar, Senegal.

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.