[Prevalence and risk factors for sickle retinopathy in a sub-Saharan comprehensive Sickle Cell Center]. / Prévalence et facteurs de risque de la rétinopathie drépanocytaire dans un centre de suivi drépanocytaire d'Afrique subsaharienne.

INTRODUCTION: Retinopathy is a chronic complication with severe functional consequences in patients with sickle cell disease. Its prevalence is not well known in sub-Saharan Africa because of the absence of screening. We report here the results of a routine screening for sickle retinopathy in a Comprehensive Sickle Cell Center in Sub-Saharan Africa. METHODS: Screening of sickle retinopathy was carried out in all sickle cell patients aged 10 and over, followed between 2010 and 2012. Retinopathy was screened by dilated indirect fundoscopic examination and retinal angiography, if necessary. The gender, age and hematological parameters of patients with sickle retinopathy were compared with those of controls randomly selected from the cohort of sickle cell patients without retinopathy followed during the same period. RESULTS: The overall prevalence of sickle cell retinopathy was 8.8% (142/1604): 12.4% (91/731) in SC, 5.2% (38/734) in SS, 9.4% (5/53) in Sß°-thalassemia patients and 9.3% (8/86) in Sß+-thalassemia patients. Proliferative retinopathy was more common in SC patients (P<0.01). High levels of hemoglobin or of hematocrit were associated with retinopathy in all patients and with proliferative retinopathy in SC patients. In SS or Sß0thalassemia patients, high leukocyte count was associated with proliferative retinopathy. Low fetal hemoglobin level was associated with retinopathy in all groups. CONCLUSION: The prevalence of sickle cell retinopathy is high and negatively associated to the level of fetal hemoglobin. The efficiency of a routine screening for sickle cell retinopathy must be assessed in Africa as well as the benefit of phlebotomy and hydroxyurea therapy as a preventive treatments.

[Screening of cerebral vasculopathy in sickle cell anemia children using transcranial Doppler]. / Dépistage de la vasculopathie cérébrale drépanocytaire par doppler transcrânien au Mali.

Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/ß(0), and 27 S/ß(+) thalassemia patients) aged 1-17 years in a comprehensive center for follow-up and research on sickle cell disease in Bamako, Mali. After exclusion of 30 inadequate results and one case of abnormal TCD observed in a multiple organ failure patient, we found an abnormal or conditional TCD in 18% of 541 children examined in a steady state. The highest prevalence of abnormal cases concerned homozygous SS patients (8.1%). No case of abnormal or conditional TCD was observed in children with S/ß(+) thalassemia. Hemoglobin concentrations were significantly lower in patients with conditional or abnormal TCD (P<0.01). In a subgroup of 68 patients with conditional TCD, nine (13%) converted to abnormal TCD over 1 year. In this subgroup of 68 conditional TCD patients, a decrease or increase in baseline hemoglobin concentration was predictive of conditional or abnormal TCD at the follow-up visit. Progression towards conditional TCD was observed in four patients (0.9%) who initially had normal TCD. Children with abnormal TCD had, whenever possible, a monthly exchange transfusion program. One case of transient stroke in the context of P. falciparum malaria with low hemoglobin concentration and one death were observed. These findings highlight the need for systematic TCD in sickle cell disease monitoring and implementing regular blood transfusion programs in the context of limited access to regular and secure blood transfusions.

[Human parvovirus B19 infection in sickle cell anemia patient in Mali: a case-control study]. / L'infection par l'érythrovirus B19 chez le drépanocytaire au Mali : une étude cas-témoins.

Human parvovirus B19 (HP-19) is the only Parvoviridae known to be pathogenic in human. Studies of HP-19 infection and its associated life-threatening complications in sickle cell anemia patients have been reported in Europe and the US. These results justify the development of HP-B19 prevention and strategies to reduce the incidence of severe and life-threatening complications associated with the infection in patients with sickle cell anemia, particularly in sub-Saharan Africa where the sickle cell anemia burden is high. In light of these considerations, we conducted a case-control study including 163 patients with sickle cell anemia and 163 controls. HP-B19 diagnosis was based on the detection of IgG and IgM antibodies specific for HP-B19 using commercially available enzyme immunoassays. Anti-human parvovirus B19 IgG antibodies were found in 105 of 193 (64.8%) patients vs 79 of 193 controls (48.4%). IgM antibodies were found at a higher frequency in sickle cell anemia patients than in controls. This higher frequency was found to be age-dependent. However, the reticulocyte count showed no significant decrease in Malian patients with sickle cell anemia. Further studies are needed to better characterize the implication of HP-B19 infection in sickle cell anemia mortality and morbidity and to develop preventive strategies and efficient management of the resulting complications.

A metastatic case of gastrointestinal stromal tumor (GIST) treated with imatinib mesylate (Glivec(®)) in Mali.

Gastrointestinal stromal tumors (GIST) usually showing a spindle cells pattern of cell proliferation have recently benefit from a molecular definition. Indeed imatinib mesylate (Gleevec(®)) treatment has dramatically improved the management of these tumors as they frequently express the c-kit oncogene. We report the first case of a metastatic gastric GIST in a man of 45 years diagnosed and treated in Mali. The gastric tumor was particularly aggressive with a large intra-abdominal and mesenteric spreading and liver metastases. The diagnosis was done on the CD117 and CD34 expression in the tumor sample obtained by laparotomy. After a 34 months 400mg/day imatinib mesylate (Gleevec(®)) treatment a dramatic tumor regression was obtained.

Facteurs limitant l'acces aux medicaments anticancereux dans un CHU de Bamako; Mali

Ce travail dont l'objectif etait d'etudier les facteurs limitant l'acces des malades aux anticancereux au Mali; a ete conduit aupres de 30 usagers du service d'hematologie oncologie medicale et de la pharmacie du CHU du Point G; ainsi qu'aupres de pharmacies privees de Bamako. Le support d'enquete etait une fiche d'enquete preetablie et mis a la disposition des personnes enquetees. Les patients ages de 4 a 60 ans; se repartissant entre 9 femmes et 22 hommes ont ete pris en charge pour un cancer du sein (19 cas); une maladie de kaposi (5 cas); une leucemie (2 cas); une maladie de Hodgkin (2 cas); une drepanocytose (1 cas) ou un CMI (1 cas). Tres peu de prescriptions ont pu etre satisfaites par l'approvisionnement hospitalier a cause d'une politique pharmaceutique particuliere du CHU. L'acces des malades aux anticancereux dans les officines privees a ete limitee par l'insuffisance des stocks d'anticancereux et des officines qui en faisaient la commande; le cout eleve des medicaments quand ils etaient commandes et les difficultes geographiques d'acces aux lieux d'achat (longues distances a parcourir). L'inscription des anticancereux sur la liste des medicaments essentiels ainsi que la mise en oeuvre de financements alternatifs pourrait permettre l'acces d'un plus grand nombre de malades aux medicaments anticancereux au Mali

[Frequency of red blood cell alloimmunization in polytransfused patients at the university teaching hospital of Point G, Bamako, Mali]. / Fréquence de l'allo-immunisation érythrocytaire chez les malades polytransfusés au centre hospitalo-universitaire du Point G, Bamako, Mali.

This prospective study conducted within 9 months period aimed to determine the frequency of red cell alloimmunization among polytransfused patients of the medical Hematology and oncology ward, and the unit of hemodialysis of the Nephrology ward at the Point-G hospital. Irregular red blood cell antibody screening and identification were performed by gel-filtration method using indirect antiglobulin test and enzymatic treated cells. We did not use saline medium. A total of 78 patients were included in this study. The mean age of the patients was 36.78±14.73 years (range: 11 and 77 years). The sex ratio was of 1.11 in favour of the women. The mean blood units transfused were 12.21±9.99 units (range: 4 and 45 units). The Rhesus phenotypes Dccee, DccEe and DCcee were most predominant, with the respective frequencies of 67.9, 15.4 and 10.3%. Kell antigen was found at a frequency of 1.28%. The total rate of red cell alloimmunization was 10.3%. There was no significant difference between the two wards. All the screened agglutinins were warm antibodies belonging to the Rhesus system: anti-E (7.7%), anti-C (1.3%) and anti-D (1.3%). Only Anti-E was present among hematologic patients. We did not find a significant link between the sex, the age, the number of blood units transfused and the positivity of the antibody screening. We conclude that the frequency of post-transfusional alloimmunization is high among polytransfused patients in Mali. A systematic antibody screening among these patients and the selection of red cells with known Rhesus/Kell phenotypes would allow an optimal blood transfusion safety.

Les hemopathies malignes de l'enfant : aspects epidemiologiques dans le service d'hematologie oncologie medicale de l'hopital du point G; Bamako; Mali (1996-2003)

Selon les statistiques des pays industrialises; les hemopathies malignes sont les cancers les plus frequents chez l'enfant. L'absence de registres specifiquement consacres a ces pathologies et le deficit d'etudes cliniques expliquent que les aspects epidemiologiques et pronostiques des hemopathies malignes de l'enfant sont mal connus dans les pays en developpement notamment ceux d'Afrique subsaharienne. Pourtant; la maitrise progressive des affections pediatriques autrefois preoccupantes; pourrait engager desormais les pays du Sud dans l'elaboration de programmes specifiques de lutte contre les hemopathies malignes de l'enfant; d'ou la necessite de donnees epidemiologiques locales. Cette etude decrit les particularites epidemiologiques des hemopathies malignes de l'enfant dans un service hospitalier de dernier niveau de reference au Mali. Cin- quante neuf cas d'hemopathies malignes de l'enfant ont ete recrutes de janvier 1996 a decembre 2003 chez 19 filles et 40 garcons. L'analyse des donnees enregistrees retrospectivement sur logiciel Access a ete faite par SPSS 11.0. L'age des enfants variait entre 4 et 15 ans avec une classe modale correspondant a 6-10 ans. Le taux de recrutement annuel etait stable sur les 8 annees considerees avec une moyenne de 7;37 nouveaux cas par an. Les hemopathies malignes predominantes etaient les lymphomes malins (70) notamment le lymphome de Burkitt. La maladie de Hodgkin n'etait pas observee avant 5 ans; mais elle representait 24des hemopathies apres cet age avec une predominance masculine. Cette etude souligne la necessite de la mise en place de strategies permettant une meilleure comprehension des aspects epidemiologiques des hemopathies malignes de l'enfant au Mali et de politiques de prise en charge et de prevention des cas