[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%).

[Evaluation of antiretroviral therapy in HIV-infected adults in the Department of Haematology, University Hospital of Brazzavllle, Congo]. / Evaluation du traitement antirétroviral chez les adultes infectés par le VIH, suivis dans le service d'hématologie du CHU de Brazzaville, Congo.

A retrospective study was conducted during 32 months; from 1 May 2003 to 30 December 2005 in haematology department. The objective of the study was to assess the effectiveness of the anti retroviral therapy 157 patients receiving antiretroviral treatment for at least a twelve month-period and presenting AIDS symptoms based on revised CDC criteria were included. The average number of initial T4 lymphocytes is 133/mm3 (extremes 1 and 385) and the initial plasmatic average viral load, quantified in 96 patients is 214,000 copies (extreme 30,000 et 999,000) The initial antiretroviral combinations were as follows: ZDV or D4T + LMV + NVP (59.2%); ZDV or D4T + LMV + EFV (28.7%), ZDV or D4T + LMV + IDNV (8.9%); ZDV or D4T + DDI + NVP (3.2%). The results of the study are: observance rate during the first 12 months (84%), antiretroviral therapy taken irregularly (10.8%), early submission of therapy (5.2%), weight gain after 24 months: +18 kgs, clinical response globally positive. The immune response is characterised by an average increase of 353/mm3 of CD4 after 24 months. Among 96 patients tested, the plasmatic viral load was undetectable in 71% of cases after a 12 month-follow up. Mild adverse drug effects have been noticed, represented by cutaneous and nervous toxicity anaemia and digestive disorders due to indinavir These therapeutic results confirm the importance of the antiretroviral therapy in the improvement of the quality of life of HIV/AIDS patients but a concern remains on the possible drug resistance still not documented.

[Etiologic profile of hyperspleenism at the University Hospital of Brazzaville]. / Bilan etiologique de l'hypersplenisme au CHU de Brazzaville.

AIM: etiologic aspect and indication of splenectomy for Hyperspleenism. METHODS: 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. RESULTS: 74 patients presenting Hypersplenism were identified, that were characterised by splenomegalia 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 spleenectomy were determined to establish the etiologic profile. Splenomegalia was often large and cytopenia was frequently severe. The profile shows a highest rate of lymphoproliferative disease (48 cases; 64.6%), including lymphomas and tricholeucocytic leukaemia. CONCLUSION: 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.

[Sudden deafness in sickle cell anemia: a case report]. / La surdité brutale chez le drépanocytaire: à propos d'un cas.

The non-expected deafness is quite obvious or easily diagnosed cause. The sickle cell disease is one of the aetiologies of this one. We present one case observed on a 30 years old patient. There is a close connection between the vascular factor of the non-expect deafness and the erythrocytic falciformation of sickle cell anaemia causing the obliteration of the terminal auditory internal artery and generating the ischaemia of the cochlea anoxia. The high sensitivity of the cochlea to anoxia and its great fragility require an early therapy in order to recover auditory capacity. Patients suffering from sickle cell disease should be encouraged to have a regular assessment of audition.

[Multiple myeloma and HIV infection: report of 3 cases]. / Myélome multiple et infection a VIH/SIDA : a propos de trois observations.

HIV infection rages at the endemic state in Sub Saharan African and especially in Congo Brazzaville. We report the observation of three female patients infected with HIV and developing multiple myeloma. The three patients were treated at the University hospital of Brazzaville between 2000 and 2002. In two cases multiple myeloma was discovered after the diagnosis of HIV infection. In the other case, the diagnosis of HIV infection was posterior to the occurrence of multiple myeloma. HIV infection was symptomatic in two cases who received consequently antiviral treatment. Multiple myeloma was diagnosed at an advanced stage in the three cases. The paraprotein was an IgG in two cases and an IgA in the other one. The CD4 counts before treatment were around 200/mm3 in two cases and within normal limits in the third case. Viral load was not measured. VMCP and VAMCP regimens were administered without major complications and under anti-infectious prophylaxis. The follow-up is still insufficient to assess the medium-term evolution and to determine the prognosis of multiple myeloma. The description of these three cases confirms the involvement of HIV in B cell lymphoma genesis.

[Multiple myeloma and HIV/AIDS infection. Three case reports]. / Myelome multiple et infection à VIH/SIDA. A propos de 3 observations.

HIV/AIDS infection rages at the endemic state in Sub Saharan African and especially in our country. We are reporting 3 observations of patients, all of females affected by HIV/AIDS and multiple's myeloma. They are all in-patients in the department of haematology, University hospital of Brazzaville, from 2000 to 2002. In two cases out of three, multiple's myeloma is discovered after the tracking of HIV/AIDS infection. In the other case, the tracking HIV/AIDS seems to be posterior to the multiple's myeloma diagnostisis. HIV/AIDS is symptomatic in the tree cases and under antiviral treatment. Multiple's Myeloma is diagnosed at an advanced stage. It is about IgG myeloma in two cases and IgA myeloma in the other. The rate of the T4 lymphocytes is noted spontaneously to 204 and 486 by mm3, and 390 by mm3 in a patient under antiviral treatment before her hospitalisation. The measure of the viral intensity is not achieved. The poly chemotherapy of type VMCP and VAMCP is driven without major complications and under anti-infectious prophylaxis. The receding is still insufficient to affirm the medium-term evolution and to determine the prognosis of the malign blood disease. The description of these three observations confirms the implication of the human retrovirus and in particular of the VIH/SIDA in the lymphoma genesis.

[Epidemiologic and cytologic configuration of acute leukaemia at University Hospital of Brazzaville]. / Configuration épidemiologique et cytologique des leucémies aiguës 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 (107%). reported to the number of inpatients admitted on a total of 52,458 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 2 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

[Blood transfusion assessment to 112 homozygous sickle-cell disease patients in university hospital of Brazzaville]. / Evaluation de la transfusion sanguine chez 112 patients drépanocytaires homozygotes au CHU de Brazzaville.

Homozygous, sickle-cell disease (SCD) is responsible for acute complication, especially anaemic crisis and special situation such as acute chest syndrome, stroke and acute priapism. Pregnancy sickle-cell disease presents high risk for the mother and the fetus. In these indications, blood transfusion is the main therapy aiming to reduce anaemia in order to restore hemoglobin's rate or to increase normal Hb proportion. This study aims to assess the short-term efficiency of the red cell transfusion in SCD homozygous form. One hundred and twelve homozygous sickle-cell patients were enrolled in this prospective study: 59 females and 53 males, median age is 21,8 years (extremes: 2 and 45 years). These patients are mostly with very low income. Two groups of patients are included in this study. In the first group, patients present acute anemia crisis caused by infections disease (malaria, bacterial infections). In the second group (20 cases), SCD patients have particularly situations: pregnancy (10 cases); stroke (six cases); cardiac failure (two cases) and priapism (two cases). Transfusion treatment in first group is simple regimen. Transfusion of EC increased median Hb level at 2,9 g/dl (extremes: 1,1 and 4,7). In the second group of patients, 16 cases were transfused by manual partial exchange (1-3) and four patients received simple regimen of transfusion. Median Hb level was 3,1g/dl (extremes: 2,4-4,9 g/dl). HbS percentage reduction was after PTE between -30 and -66,8% (median: -52,6%). According to our diagnostic possibilities (blood serologic test), we have not found any contamination by HIV, HBV and HCV (virus).