[Sexuality assessment of homozygous adult sickle cell patients with a history of priapism in Senegal]. / Évaluation de la sexualité des drépanocytaires adultes homozygotes ayant des antécédents de priapisme au Sénégal.

OBJECTIVES: The objective of this study was to evaluate the sexuality of SS sickle cell patients with a history of priapism. METHODS: This was a case-control study of adult SS sickle cell patients. The occurrence of priapism as well as the nature of the priapism had been investigated. The patients were subdivided into three groups: Group 1 (no priapism), Group 2 (intermittent priapism) and Group 3 (acute priapism). The patients' sexuality was studied using the IIEF-15 questionnaire. RESULTS: We interviewed 191 SS sickle cell patients. The mean age was 27.1±7.1 years. Priapism was observed in 43.5 %. Only 77 patients were eligible for the IIEF15 questionnaire. Groups 1 and 2 performed significantly better than group 3 on erectile function (EF) and orgasmic function (OF) scores. There was no significant difference in the EF and OF scores between groups 1 and 2. No significant difference was observed between the three groups for the scores of sexual desire (SD), intercourse satisfaction (IS), and overall satisfaction (OS). The impairment of erectile function in group 2 was related to the age of the first episode of priapism and the last episode. The impairment of erectile function in group 3 was related to the duration of evolution (P<0.05). CONCLUSION: This study shows that priapism is responsible for impaired erectile function in SS adult sickle cell patients. A program to prevent intermittent episodes of priapism should be put in place.

Diagnosis Dilemma of Angioimmunoblastic T-Cell Lymphoma in Tuberculosis Endemic Region.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare hematologic malignancy recognized in the WHO 2016 classification as a clinical and histological entity. It is a very poorly described disease in Africa due to its rarity and diagnostic difficulties, particularly differential diagnosis with tuberculosis. Here, we report a 57-year-old man who presented with fever, weight loss, and lymphadenopathies. The diagnosis of tuberculosis was carried out based on lymph node fine needle aspiration showing the image of tuberculous adenitis and CT images in favor of necrotic lymphadenopathies. The presence of autoantibodies and the failure of tuberculosis treatment led us to perform a biopsy with immunostaining that confirmed pathological features of AITL. The patient was treated by CHOP-based chemotherapy, and complete remission was achieved. This case highlights the difficulty of recognizing AITL and the importance of considering other potential differential diagnoses of tuberculosis in the endemic region.

Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country.

INTRODUCTION: The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. PATIENTS AND METHOD: Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. RESULTS: We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/ß0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was -60%. Neither alloimmunization nor viral seroconversion was observed. CONCLUSION: This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

[Isolated microspherophakia in a Senegalese family]. / Microsphérophakie isolée dans une famille sénégalaise.

PURPOSE: To report the clinical investigation of isolated microspherophakia involving a Senegalese family in order to appreciate its functional impact. OBSERVATIONS: This is a rural family comprised of 7 members. The sibship included three girls and two boys. One of the girls, who lived in a distant zone, was unable to be examined. Of all the examined members of the family, only the father was unaffected by the illness. There was no consanguinity. The general medical examination was normal. The best-corrected visual acuity (VA) for the girls was 2/10. For one of the boys, BCVA was 8/10 for both eyes, and for the other, BCVA was 10/10 for the right eye and 8/10 for the left eye. The mother's VA was 10/10 and P2 without correction. Myopia and astigmatism were present in the 4 children of the sibship. During the examination, we noted the presence of small crystalline lenses, which were very round and presented an abnormal visibility of the lens equator and zonular fibers. The diagnosis of microspherophakia was confirmed by measurement of the lens diameters by ultrasound biomicroscopy. Complications were present in the girls, including pupillary block glaucoma and amblyopia for the elder, and retinal degeneration and amblyopia for the younger daughter. The elder daughter was managed medically with glaucoma drops. The younger daughter received optical correction and a prophylactic Argon LASER treatment. The two boys received optical correction. No treatment was recommended for the mother. CONCLUSION: Microspherophakia is a rare condition. Some serious complications can lead to amblyopia. A better multidisciplinary evaluation would allow for early detection and a better prognosis.

[Profil course of SC sickle-cell patients in Dakar: a case-control study with SS sickle cell form]. / Profil evolutif de la drepanocytose SC A Dakar: étude cas-témoin avec la forme SS.

AIMS: The study aim to describe the epidemiological, clinico-biological and evolutionary aspects of SC sickle cell patients compared to SS sickle cell disease. PATIENTS AND METHODS: We realized a case-control study of 12 months duration including 98 major sickle cell patients (49 SC and 49 SS sickle cell patients). SS sickle cell patients were randomly selected according to age and sex. Socio-demographic, clinico-biological and evolutionary data were noted for each patient. RESULTS: Mean age was 24.7 years (5 - 53). Sex ratio was 0.8. Mean number of transfusions was 0.06 of SC patients and 0.34 for SS patients (p=0.0008). Mean number of vaso-occlusive crisis per year was 2.24 of SC patients and 2.37 of SS patients (p=0.3). Mean basic hemoglobin level was 10.8 of SC patients and 7.8 of SS patients (p=0.0000). Priapism was found in 2.04% of SC patients and 4.04% of SS patients (p=0.3) and acute anemia in 2.04% of SC and 24.48% of SS patients (p=0.003); 26.53% of SC patients had a chronic complication compared to 18.36% of SS patients (p=0.0001). CONCLUSION: This study shows that SC sickle cell patients are less symptomatology compared to SS patients, however they would develop more chronic complications from where the utility for regular follow-up.

BUTS: Le but de notre étude était de décrire les aspects épidémiologiques, clinico-biologiques et évolutifs des patients SC comparés aux patients SS. PATIENTS ET MÉTHODES: Il s'agissait d'une étude cas témoins d'une durée de 12 mois portant sur 98 patients (49 SC et 49 SS8). Les témoins SS étaient choisis de façon aléatoire après appariement selon l'âge et le sexe. Pour chaque patient nous avons noté les aspects sociodémographiques, clinicobiologiques et évolutifs. RÉSULTATS: L'âge moyen était de 24,7 ans (5 - 53). Le sex ratio était de 0,8. Le nombre moyen de transfusions était de 0,06 chez les SC et 0,34 chez les SS (p=0,0008). Le nombre moyen de CVO/an était de 2,24 chez les SC et 2,37 chez les SS (p=0,3). Le taux moyen d'hémoglobine de base était de 10,8 chez les SC et 7,8 chez les SS (p=0,0000). Le priapisme était trouvé chez 2,04% des SC et 4,04% des SS (p=0,3) et l'anémie aigue chez 2,04% des SC et 24,48% des SS (p=0,003); 26,53% des SC souffraient d'une complication chronique contre 18,36% des SS (p=0,0001). CONCLUSION: Cette étude montre que les drépanocytaires SC ont une symptomatologie moindre par rapport aux patients SS, cependant ils développeraient plus de complications chroniques d'où l'utilité d'un suivi régulier.

Syndrome d'activation macrophagique secondaire chez l'enfant. Rapport de 5 cas sénégalais

Le Syndrome d'Activation Macrophagique (SAM) est défini comme la traduction clinico-biologique d'une prolifération et d'une activation non-spécifique des macrophages du système réticulo-histiocytaire avec phagocytose des éléments figurés du sang. Nous rapportons 5 cas de SAM secondaires chez des enfants hospitalisés dans le service de pédiatrie de l'hôpital Aristide le Dantec entre août 2015 et avril 2016. Il s'agissait de 3 filles et 2 garçons âgés de 7 ans à 14 ans. Cliniquement, la fièvre, l'altération de l'état général et la splénomégalie étaient constantes. Quatre patients ont présenté des adénopathies et chez 2 patients une hépatomégalie a été retrouvée. Au niveau de l'hémogramme, l'anémie était constante, la thrombopénie et la leuco-neutropénie étaient retrouvées chez 3 patients et le frottis sanguin révélait 36% de blastes chez un patient. L'hémophagocytose médullaire était retrouvée chez tous les patients, l'hyper ferritinémie était constante et chez trois patients une hypertriglycéridémie avec un taux élevé de lactate déshydrogénase (LDH) ont été notés. Le diagnostic était surtout guidé par le médullogramme et basé sur les critères de l'hemophagocytic histiocytosis et les étiologies étaient infectieuses et néoplasiques. Chez trois patients, le SAM était d'origine infectieuse et les germes retrouvés étaient le Streptococcus, l'Escherichia coli et le Mycobacterium tuberculosis alors que les deux autres cas étaient d'origines néoplasiques secondaires à une leucémie aiguë myéloïde et à un lymphome hodgkinien. Le traitement était basé sur l'antibiothérapie (cas 1 et 2), les antituberculeux (cas 3) et la chimiothérapie (cas 4 et 5). L'évolution était favorable chez tous nos patients

[Evaluation of the efficacy of medical screening of blood donors on preventing blood transfusion-transmitted infectious agents]. / Évaluation de l'efficacité de la sélection médicale des donneurs de sang dans la prévention des agents infectieux.

BACKGROUND: The aim of this study was to evaluate the efficacy of medical screening to retain blood donors in window period by comparing the seroprevalence of infectious agents (HIV, hepatitis B and C, syphilis) in deferred versus accepted blood donors. MATERIALS AND METHODS: This prospective and transversal study was performed during 4 months in the National Blood Transfusion Center in Dakar (Senegal). We conducted a convenience sampling comparing the seroprevalence of infectious agents (HIV, HBsAg, HCV and syphilis) in deferred versus accepted blood donors after medical selection. RESULTS: In total, 8219 blood donors were included. Medical selection had authorized 8048 donors (97.92%) and deferred donors were 171 (2.08%). The prevalence of HIV was higher in the deferred than in accepted blood donors (1.75% vs. 0.05%) (P=0.0003; OR=35.91), as well as for HBsAg (12.87% vs. 7.35%) (P=0.006; OR=1.86). HCV antibodies were present in 0.71% of accepted blood donors and 0.58% in deferred blood donors (P=0.65; OR=0.82). Only accepted donors had brought the infection of syphilis (0.34%) (P=0.56; OR=0). CONCLUSION: Medical selection is efficient to exclude blood donors at high risk of HIV transmission and to a lesser extent of HBV. However, current medical screening procedures do not allow us to exclude donors asymptomatic carriers of HCV and syphilis.