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Constitutive hemorrhagic diseases that affect primary haemostasis are reportedly rare in sub-Saharan Africa.This study arrived to report within a Congolese family five cases of Glanzmann's thromboasthenia.5 cases of the congenital form of Glanzmann's thromboasthenia were depicted 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. Serologic analysis showed this patient to be positive for hepatitis C virus antibodies.This first description of Glanzmann's thrombo-asthenia in Blacks in sub-Saharan Africa shows the necessity of establish inter-hospital cooperation for the improvement of the management of constitutive hemorrhagic diseases in the Hematology wards.

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).

The authors report a peculiar form of Hodgkin's disease that is associated with Epstein-Barr virus infection. A 16-year-old patient was admitted for an autoimmune haemolytic anaemia associated with IgG auto-immune antibodies, linked to general clinical complications, cervical adenopathies and splenomegalia. The patient underwent hemicavectomy that was not histologically investigated. The ganglionar exeresis helped to establish the histological diagnosis of Hodgkin's disease, classic form with nodular sclerosis. Tumour cells were CD30 CD15 positive and neighbouring lymphoid cells were CD3 and CD20 positive. In situ hybridation allowed the detection of Epstein-Barr virus in tumour cells. Under ABVD chemotherapy supplemented with acyclovir and initial corticotherapy, mid-treatment evolution appears to be favourable. This observation is consistent with the hypothesis that tumour cells are of B cell origin during Hodgkin's disease, together with the involvement of Epstein-Barr virus in lymphomagenesis.