Les Complications Osseuses au Cours de la Drepanocytose au Service d’Hematologie-Oncologie du CHU de Donka de Conakry

Introduction: Sickle cell disease has an evolution characterized by acute and chronic complications that affect almost all the target organs of the body (bones, eyes, heart, kidneys, brain ...). The purpose of this study was to describe the bone complications of sickle cell disease. Patients and Methods: This was a retrospective study performed in the HematologyOncology Department of the Donka Teaching Hospital over a five-year period (5 years) from 1 January 2011 to 31 December 2015. Results: The frequency of bone complication of sickle cell disease was 15%. There was a predominance of the female sex with the sex ratio of 0.8. The average age of the patients was 27.5 years with extremes of 10 and 50 years. The main reason for consultation was bone pain found in 100% of our patients. Osteomyelitis was the most frequent 55.56%, followed by Necrosis of the femoral head 44.44%. Conclusion: The study found that the prevalence of bone complications is not negligible. A prospective and analytical study with a larger sample would be required to identify risk factors and develop a management protocol.Introduction : la drépanocytose a une évolution caractérisée par des complications aiguës et chroniques qui intéressent presque tous les organes cibles de l’organisme (os, yeux, cœur, reins, cerveau …). L’objectif de cette étude était de décrire les complications osseuses de la drépanocytose. Patients et Méthodes : Il s’agissait d’une étude rétrospective réalisée au service d’Hématologie – Oncologie du CHU de Donka qui s’est étalée sur une période de cinq ans (5ans) allant du 1 er janvier 2011 au 31 décembre 2015 Résultats : La fréquence des complications osseuses de la drépanocytose était de 15%. Il y avait une prédominance du sexe féminin avec le sex-ratio de 0,8. L’âge moyen des patients était de 27,5 ans avec des extrêmes de 10 et de 50 ans. Le principal motif de consultation était la douleur osseuse retrouvée chez 100% de nos patients. L’ostéomyélite était la complication la plus fréquente soit 55,56 % suivie de la nécrose de la tête fémorale soit 44,44%. Conclusion : Il est ressorti de l’étude que la prévalence des complications osseuses est non négligeable. Une étude prospective et analytique avec un échantillon plus élevé serait nécessaire pour identifier les facteurs de risque et élaborer un protocole de prise en charg

Un Cas de Lupus Erythemateux Dissemine (LED) Revele par une Anemie Chronique au Service d’hematologie Clinique du CHU de Yopougon

The authors report one case of systemic lupus erythematosus revealed by chronic anemia. This was a 29-year-old patient with long-term fever, chronic skin and joint lesions with isolated hypochrome microcytic haemolytic anemia on the hemogram. The diagnosis of SLE was made three years after the onset of symptomatology based on seven of the American Rheumatology Association's (ARA) criteria out of 11, including positive immunological status (antinuclear antibodies and native DNA). This observation shows the interest of evoking SLE, while looking for signs in a young woman with multiple and varied symptoms with signs of skin, kidney, osteoarticular and hematological disorders.Les auteurs rapportent un cas de lupus érythémateux disséminé révélé par une anémie chronique. IL s’agissait d’une patiente de 29 ans présentant une fièvre au long cours, des lésions cutanées et articulaires d’évolution chronique avec à l’hémogramme une anémie hémolytique isolée hypochrome microcytaire. Le diagnostic de LED a été retenu trois années après le début de la symptomatologie devant sept critères sur 11 de L’American Rheumatology Association (ARA) dont le bilan immunologique positif (anticorps antinucléaires et DNA natif). Cette observation montre l’intérêt d’évoquer le LED, tout en recherchant les signes chez une femme jeune présentant une symptomatologie multiple et variée avec les signes d’atteintes cutanée, rénale, ostéo-articulaire et hématologique

Extent and Dynamics of Polymorphism in the Malaria Vaccine Candidate Plasmodium falciparum Reticulocyte-Binding Protein Homologue-5 in Kalifabougou, Mali

Reticulocyte-binding homologues (RH) are a ligand family that mediates merozoite invasion of erythrocytes in Plasmodium falciparum. Among the five members of this family identified so far, only P. falciparum reticulocyte-binding homologue-5 (PfRH5) has been found to be essential for parasite survival across strains that differ in virulence and route of host-cell invasion. Based on its essential role in invasion and early evidence of sequence conservation, PfRH5 has been prioritized for development as a vaccine candidate. However, little is known about the extent of genetic variability of RH5 in the field and the potential impact of such diversity on clinical outcomes or on vaccine evasion. Samples collected during a prospective cohort study of malaria incidence conducted in Kalifabougou, in southwestern Mali, were used to estimate genetic diversity, measure haplotype prevalence, and assess the within-host dynamics of PfRH5 variants over time and in relation to clinical malaria. A total of 10 nonsynonymous polymorphic sites were identified in the Pfrh5 gene, resulting in 13 haplotypes encoding unique protein variants. Four of these variants have not been previously observed. Plasmodium falciparum reticulocyte-binding homologue-5 had low amino acid haplotype (h = 0.58) and nucleotide (π = 0.00061) diversity. By contrast to other leading blood-stage malaria vaccine candidate antigens, amino acid differences were not associated with changes in the risk of febrile malaria in consecutive infections. Conserved B- and T-cell epitopes were identified. These results support the prioritization of PfRH5 for possible inclusion in a broadly cross-protective vaccine

Subclinical Cardiac Dysfunction Is Associated With Extracardiac Organ Damages

Background: Several studies conducted in America or Europe have described major cardiac remodeling and diastolic dysfunction in patients with sickle cell disease (SCD). We aimed at assessing cardiac involvement in SCD in sub-Saharan Africa where SCD is the most prevalent.Methods: In Cameroon, Mali and Senegal, SCD patients and healthy controls of the CADRE study underwent transthoracic echocardiography if aged ≥10 years. The comparison of clinical and echocardiographic features between patients and controls, and the associations between echocardiographic features and the vascular complications of SCD were assessed.Results: 612 SCD patients (483 SS or Sβ0, 99 SC, and 19 Sβ+) and 149 controls were included. The prevalence of dyspnea and congestive heart failure was low and did not differ significantly between patients and controls. While left ventricular ejection fraction did not differ between controls and patients, left and right cardiac chambers were homogeneously more dilated and hypertrophic in patients compared to controls and systemic vascular resistances were lower (p < 0.001 for all comparisons). Three hundred and forty nine SCD patients had extra-cardiac organ damages (stroke, leg ulcer, priapism, microalbuminuria or osteonecrosis). Increased left ventricular mass index, cardiac dilatation, cardiac output, and decreased systemic vascular resistances were associated with a history of at least one SCD-related organ damage after adjustment for confounders.Conclusions: Cardiac dilatation, cardiac output, left ventricular hypertrophy, and systemic vascular resistance are associated with extracardiac SCD complications in patients from sub-Saharan Africa despite a low prevalence of clinical heart failure. The prognostic value of cardiac subclinical involvement in SCD patients deserves further studies

INTEREST IN DETERMINING THE CD34+ CD38- PHENOTYPE IN THE DIAGNOSIS AND PROGNOSIS OF ACUTE LEUKEMIA IN ABIDJAN – CÔTE D’IVOIRE

Background In Côte d’Ivoire, acute leukemias account for 12.5% of hematological malignancies. Acute leukemias are due to an anomaly of the stem cell characterized among other things by the expression of CD34+ CD38- surface markers. This CD34+ CD38- phenotype as well as other factors such as tumor syndrome, high leukocytosis and blasts are considered as important factors of poor prognosis. We therefore proposed to investigate the prognostic value of the expression of CD34+ CD38- markers in acute leukemias in Abidjan. Methods We selected 23 patients aged 33 years on whom we performed Complete Blood Count, bone marrow aspiration and immunophenotyping. To search for myeloperoxydase, smears of blood or bone marrow were stained with benzidine and revealed by the use of Hydrogen peroxide. Acute leukemias were then identified and distributed using the score proposed by the European Group for the Immunological characterization of Leukemias. The definitive diagnosis was made by combining morphological characters that serve as the basis for the French-American-British classification as well as cytochemical and immunophenotypic characters. Results According to the cytological and immunophenotypic classifications, the acute lymphoid leukemia 2 and B IV predominated. 52.2% (12/33) of patients were CD34+ CD38-. This phenotype was found in almost all cytological immunophenotypic types. The medullary invasion by blasts (reflection of the tumor mass) of the total sample of CD34+ , CD34+ CD38- patients and those not expressing CD34+ was respectively 79.4%, 81.25%, 83.3% and 74.8%. Conclusion There was therefore no correlation between medullary blasts and the expression of CD34+ CD38-. To the factors we selected it would have been necessary to associate the study ofcytogenetic and molecular anomalies to better understand the role of CD34+ CD38- phenotype, concerning prognosis

Hypercalcémie importante révélant un myélome multiple chez une adolescente de 19 ans

Le myélome multiple est caractérisé par une prolifération des plasmocytes malins sécrétant une immunoglobuline monoclonale complète ou incomplète. C’est une affection de l’adulte mûr. L’âge moyen de survenue est au-delà de 50 ans, rare avant 40 ans et exceptionnel chez les enfants ce qui fait qu’il n’est pas toujours évoqué en première intention chez les sujets jeunes en Afrique Noire. Nous rapportons dans cette étude, l’observation d’une adolescente de 19 ans, sans antécédents pathologiques particuliers, adressée dans notre service pour l’investigation d’une anémie sévère. La symptomatologie était dominée par une hypercalcémie importante inexpliquée associée à une insuffisance rénale. Le myélogramme fait dans le compte d’une anémie normochrome normocytaire arégénérative notait une infiltration médullaire d’environ 12 % par les plasmocytes dysmorphiques. La présence d’une immunoglobuline monoclonale IgG exprimant la chaine légère de type kappa à l’immunofixation des protéines urinaires et les lésions osseuses multiples ont permis de porter le diagnostic de myélome multiple. La chimiothérapie par le protocole VRD a permis une rémission partielle avec correction de la calcémie. L’intérêt de cette étude réside dans le caractère exceptionnel de cette affection à cette tranche d’âge suscitant un intérêt étiopathogénique. Ceci devait motiver les praticiens à y penser devant les signes révélateurs chez les sujets jeunes.Mots-clés: Myélome multiple, adolescent, AbidjanEnglish Title: Significant hypercalcaemia revealing multiple myeloma in young girls of 19-year-oldEnglish AbstractMultiple myeloma is characterized by proliferation of malignant plasma cells secreting complete or incomplete monoclonal immunoglobulin. It is an affection of the elderly. The average age of onset is beyond 50 years old, rare before 40 years old and exceptional in children so that it is not always evoked in first intention in young people in Black Africa. We report, the case of the girl who was 19 years old, with no particular medical history, adressed for the investigation of severe anemia. The symptomatology was dominated by unexplained significant blood calcemia associated with renal failure. The bone marrow exam, done because of an aregenerative normocytic normochromic anemia noted an infiltration about 12% of dysmorphic plasma cells in the bone marrow. The monoclonal IgG immunoglobulin expressing the kappa light  chain found during the urinary proteins immunofixation and multiple bone lesions confronted to the diagnosis of multiple myeloma. The chemotherapy with the VRD protocol allowed a partiel remission with correction of the calcemia. The interest of this study resides in the exceptional character of the affection with this age group and which arouses an etiopathogenic interest. This should motivate practitioners to think about it during signs in young people.Keywords: Multiple myeloma, young people, Abidja