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[Panorama of congenital disorders of hemostasis diseases at University Hospital of Brazzaville, Congo].

Congenital disorders of hemostasis constitutes a group of affections that rarely occur in Sub-Saharan Africa. Many cases are overlooked due to insufficient laboratory facilities. The purpose of this retrospective study carried out between January 1998 and December 2006 was to collect epidemiological, laboratory, and clinical data on the various congenital disorders of hemostasis observed in the hematology department of the University Hospital of Brazzaville, Congo. A total of 42 patients ranging in age from 2 to 54 years (mean, 15.8 years) were diagnosed during the study period. There were 29 men and 13 women representing all ethnic groups in the Congo including 16 Kongos (38.1%), 12 Ngala (28.6%), 11 Tekes (26.2%) and 3 foreigners (7.1%). Cases involved all social and economic levels of society. Presenting symptoms leading to discovery of the hemostasis disorders were post-traumatic cutaneous hemorrhagic in 13 cases (30.9%), hematoma in 8 cases (19%), perioperative hematoma in 7 cases (16%), perioperative hemorrhage in 7 cases (16%), hemarthrosis in 6 cases (14.3%); spontaneous epistaxis in 5 cases (11.9%), findings during routine checkup in patients with a family history in 3 cases (7.1%), and genital hemorrhage in 1 case (3.8%). The targeted laboratory routine used for diagnosis included the following tests: bleeding time (IVY), cephaline activated time, serum assays for deficient factors, and confirmation by flow cytometry. The diagnosis was Von Willebrand disease in 20 cases (47.6%), hemophilia A in 16 cases (38.1%), Glandzman thrombasthenia in 5 cases with high consanguinity (11.9%), and factor VII deficiency in 1 case (2.4%).