[Glanzmann's thrombasthenia: first case descriptions in sub-saharan africa]

Constitutive hemorrhagic diseases that affect primary haemostasis are reportedly rare in sub-Saharan Africa This study arrived to report within a Congolese family live cases of Glanzmann's thromboasthenia 5 cases of the congenital form of Glanzmann's thromboasthenia were depictecd in a Congolese family. The disease was first discovered with a young student who was transferred in France who had shown a tendency to develop hemorrhages since childhood. This tendency was enhanced following abdominal surgery to treat peritonitis. Like the other 3 cases, she had a prolonged bleeding time, albeit with normal von Willebrand factor plasma values. A 7 year old girl died following appendectomy from post-surgery hemorrhages. In this young patient, platelet aggregation could be induced only by ristocetine all other conventional agonists failed. Flow cytometric analysis showed the total absence of GPIIbIIIa. The hemorrhages in the girls could be managed by cyclic administration of oestrogens and iron supplementation .Serological analysis showed this patient to be Positive for hepatitis C virus antibodies. This first description of Glanzmann's thromobo - asthenia in Blacks in sub-Saharan Africa shows the necessity of establish inter-hospital cooperationfor the improvement of the management of constitutive hemorrhagic diseases in the Hematology words

[Etiologic profile of hyperspleenism at the University Hospital of Brazzaville]

Etiologic aspect and indication of splenectomy for Hypersplenism. A retrospective survey was performed at the haematology ward of the University Hospital of Brazzaville on files that were compiled from January 1st, 1994 to December 31st, 2004. 74 patients presenting Hypersplenism were identified, that were characterised by splenomegaly associated with multiple cytopenia of blood cells. This list of patients included 41 female and 33 male, with an overall average age of 34 years. The etiologic profile is established based on clinical and laboratory parameters. Thus, haemograms, liver enzymes, microbiology, histopathology, including or not splenectomy were determined to establish the etiologic profile. Splenomegaly was often large and cytopenia was frequently severe. The profile shows a highest rate of Iymphoproliferative disease [48 cases 64,6%]. including lymphomas and tricholeucocytic Ieukaemia. Cirrhoses, hemoglobinopathies, and HIV infections were among the other causes of the disease in spite of the disease. In spite of the risk associated with splenectomy, this surgical procedure enabled to establish diagnosis and allowed correction of the Hyperspleenism. In conclusion, all splenomegalia associated with blood cytopenia should always submitted to exploratory scrutiny

Evaluation des effets cliniques de l'hydroxyuree chez 132 patients drepanocytaires homozygotes

Hydroxyurea is used since close to 10 years in the treatment of major sickle cell anemia. In African sub Sahara, very little study have been dedicated. Yet, this drug represents nowadays, an alternative to the bone marrow transplantation in the treatment of this illness. This study permits to illustrate the beneficial effects of this drung on 132 patients affected by of major sickle cell anemia. It is about patients whose middle age is of 28 years [range 14 and 54 years], most frequently masculine sex, followed regularly in the service of haematology of CHUB. The treatment consisted to the administration of the hydroxyurea to the posology between 10 and 30 mg/kg per day. The essentially clinical assessment focused on the sickle cell anemia crises and the modifications of hematologic's parameters. The experimentation has been pursued beyond the 18th month among 108 patients. It takes out again of this survey disappearances of the vasculo occlusive crises among 103 patients on 132 [78.03%] during the first 18 months of the survey. Only 10 patients [7.57%] benefited blood transfusion during the period of the survey because of the infectious complications. The hydroxyurea led a meaningful and spontaneous increase of the haemoglohin rate [+2.54 g/dl] of the middle globular volume [+ 16.02 femtolitres] as well as of the rate of haemoglobin foetale on 65 estimated patients. The hydroxyurea didn't bring any objective improvement on the pre-existing degenerative complications. The treatment has been interrupted precociously among 5 patients for cytopenia. This survey confirms to short and middle term the beneficial effects of the hydroxyurea on the major sickle cell anaemia

Configuration epidemiologique et cytologique des leucemies aigues au centre hospitalier et universitaire de Brazzaville

The authors report the epidemiologic and cytological aspects of 77 patients hospitalized between January 1995 and December 2002 in the clinical hematologic service in university hospital complex of Brazzaville, greatest hospital of the country. During this period, 7155 patients were hospitalized in this service as a frequency of [1.07%]. Reported to the number of inpatients admitted on a total of 52458 patients. The hospital frequency of disease is 0.15%. There's no specific age for this affection [age median =21-24 years] and all socio-professional categories can be affected with a prevalence of low income patients what complicates extremely the treatment. Ratio man woman is 0.8 showing a light female prevalence. On the level of the epidemiologic investigation [limited because of the insufficiency of the means], nothing is retained except rare acute leukaemias secondary to chronic myeloproliferative disorders as well as association with 02 cases of homozygous sickle cell anaemia. Mortality by acute leukaemia in the service is very high, due to lack of equipment. In the cytological plan, there's a light prevalence of the cases of acute lymphoblastic leukaemias of which some could profit from an immunological typing compared to the cases of myeloblastic acute leukaemias