[Results of partial transfusion exchange in 42 homozygous sickle cell patients at university hospital of Brazzaville]. / Résultats des échanges transfusionnels partiels chez 42 patients drépanocytaires homozygotes au CHU de Brazzaville.

The standard of care for major sickle cell diseases in crisis is based on blood transfusion, but this remains a risky therapy in sub-Saharan Africa. The objective of this retrospective and prospective study was to assess exchange transfusion (ET) in homozygous sickle cell disease between 1st July 2005 and 30th June 2008 at the transfusion centre of university hospital of Brazzaville. The ET technique used was manual and made of three stages: bleeding, infusion of solution, and infusion of red cell concentrate. Clinical and biological assessments were done before and after exchanges. The indication for ET were: pregnancy (19 cases); strokes (seven cases); vaso-occlusive crisis (five cases), priapism (four cases), cardiac failure (three cases) and miscellaneous (four cases), the values of hematocut in red cell blood bag between 0.55 and 0.70 (median 0.65). The median haemoglobin level before exchange was about 5.8 g/dl (4.1-7.4 g/dl), that of HbS from haemoglobin electrophoresis about 98.4 % (94.6-100 %). Before the exchanges, viral serological tests were done with the following results: two patients with HBV antigen positive and one patient antibodies to HCV. In acute situation interval between exchanges is 3 to 10 days, whereas during chronic situation is between 14 to 28 days. No major immediate complications have been observed. Clinical situation after exchange characterized by stabilization of cerebral vascular stroke and over and control of one case of priapism complications. Assessment after exchange showed the following results: medium rate of Hb level: 9.5 g/dl (an increase of 3.7 g/dl); medium percentage of HbS reduced to 46.8 % (approximately a decrease of 51.6 %). Patient's serological status to HCV changed for one patient. This study illustrates the benefit and the limitations of the transfusion exchange during sickle cell disease in sub-Saharan Africa.

[Panorama of congenital disorders of hemostasis diseases at University Hospital of Brazzaville, Congo]. / Maladies hémorragiques congnitales au Congo. Expíence du service d'hématologie du centre hospitalier et universitaire de Brazzaville.

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%).