Therapeutic phlebotomy during major sickle cell disease in Togo.

To determine the influence of therapeutic phlebotomy on the reduction of vaso-occlusive crises (VOCs) and of hospital admissions of patients with sickle cell disease and to describe how they experience this practice. Descriptive cross-sectional study of 27 patients with sickle cell disease who underwent phlebotomies. We estimated the number of VOCs, hemoglobin levels, and patient response. Among 27 sickle cell patients (24 SC and 3 SS) who underwent phlebotomies, there were 22 men (81.5 %) men and 5 (18.5 %) women, with an average age of 34.6 ±10.9 years (range: 21-56 years). Before the phlebotomies, 23 (85.2 %) had showed signs of hyperviscosity, they had a mean of 5.3 ± 1.02 (range: 3-8) VOCs annually, and their mean hemoglobin level was 14.3 ±1.5g/dl (range: 10.4 g/dl-16.8 g/dl). The mean number of phlebotomies was 4.9±4.11 (range: 1-13). After this treatment, 21 (91.3 %) reported that their signs of hyperviscosity disappeared, and the mean number of VOCs annually decreased to 0.9 ±0.07 (range: 0-2). The mean change in hemoglobin was 1.9 ±0.8 g/dl (range: 0.9-3.2 g/dl). Nine patients (6SC and 3SS), 7 under anti-anemic treatment, initially refused phlebotomy, mainly because they did not understand how it could be useful to reduce the amount of blood in patients with a potentially anemic disease. Phlebotomy has resulted in a decrease in the frequency of VOCs and hospitalizations. It is essential to further popularize it and increase patients' aware of its value in combatting sickle cell disease and thus improve their willingness for and adherence to this treatment.

[Biermer's disease in the hematology service of campus teaching hospital of Lomé: 13 cases from January 2013 to December 2014]. / La maladie de biermer, a propos de 13 cas colliges du 1er Janvier 2003 au 31 Decembre 2014 au CHU campus de Lome.

AIMS: To study the epidemiological, clinical, paraclinical and therapeutic aspects of Biermer's disease in the hematology service of campus teaching hospital of Lomé. METHOD: This retrospective study was conducted in the laboratory service of the University Hospital campus in which there is a unit of clinical hematology. This study took place over a period of twelve years and has identified 13 patients (5 men, 8women). Patients were selected on the basis of clinical, paraclinical (blood cells count, bone marrow, dosage of vitamin B12 and the intrinsic factor antibody) and the therapeutic test. RESULTS: The mean age was 53 years ranging from 32 to 68 years. There was a female predominance with a sex ratio of 0.6. Clinically, there was conjonctival palor and asthenia (92% each) followed by paresthesia (62%). The blood cell count essentially showed profound anemia (7g/dl on average), macrocytic (84,6%), normocytic (15,3%), bicytopenia (61,5%) and pancytopenia (23% ). In all patients, the bone marrow study showed megaloblasts, the dosage of the vitamin showed a collapse of vitamin B12 (76,9%), and intrinsic factor antibody was positive (92,3%). The fibroscopy showed atrophic gastritis (76,9%), intestinal metaplasia (69,2%) and an absence of Helicobacter pylori. The evolution was favorable with parenteral B12 vitamin therapy for all patients. CONCLUSION: We recommend looking for iron deficiency during the follow-up and gastric biopsy even if no macroscopic lesions are found.

OBJECTIF: Etudier les aspects épidémiologiques, cliniques, paracliniques et thérapeutiques de la maladie de Biermer dans le service d'hématologie du CHU Campus de Lomé. MÉTHODE: Il s'agissait d'une étude rétrospective réalisée dans l'unité d'hématologie clinique du service des laboratoires du CHU Campus de Lomé. Cette étude s'est déroulée sur une période de douze ans et a permis de recenser 13 patients (5 hommes et 8 femmes). Les patients ont été retenus sur la base des arguments cliniques, paracliniques (hémogramme, myélogramme, dosage de la vitamine B12 et la recherche des anticorps anti-facteur intrinsèque) et le test thérapeutique. RÉSULTATS: L'âge moyen des patients était de 53 ans avec des extrêmes de 32 et 68 ans. Il y avait une prédominance féminine avec une sex-ratio de 0,6. Le tableau clinique était dominé par la pâleur conjonctivale et l'asthénie (92%) suivies de la paresthésie (62%). L'hémogramme avait montré essentiellement une anémie profonde (7g/dl en moyenne) macrocytaire (84,6%), normocytaire (15,3%), une bicytopénie (61,5%) et une pancytopénie (23%). Chez tous les patients, le myélogramme montrait des mégaloblastes, le dosage vitaminique montrait un effondrement de la vitaminémie B12 (76,9%), et l'anticorps anti-facteur intrinsèque était positif (92,3%). La fibroscopie digestive haute associée à l'examen anatomo-pathologique avait montré une gastrite atrophique (76,9%), une métaplasie intestinale (69,2%) et une absence d'hélicobacter pylori. L'évolution a été favorable sous vitaminothérapie B12 par voie parentérale chez l'ensemble des patients. CONCLUSION: Nous recommandons la recherche d'une carence martiale au cours de l'évolution et la biopsie gastrique même en l'absence de lésion macroscopique à la fibroscopie digestive haute.

[Rare hemoglobins in Togo: a 15-year study at the Lomé University Campus Hospital Center]. / Les hémoglobines rares au Togo : à propos d'une étude réalisée sur quinze ans au CHU Campus de Lomé (Togo).

OBJECTIVES: To identify the rare hemoglobins (Hbs) encountered in Togo and characterize their geographical and ethnic distribution and hematologic expression. MATERIAL AND METHOD: This retrospective descriptive study covers 27,530 hemoglobin electrophoresis tests performed in the department of Hematology of the Lomé Campus teaching hospital from January 1996 through December 2010, the national reference laboratory. The anomalies identified were studied with both alkaline and acid electrophoresis and high performance liquid chromatography (HPLC), all performed in Lome. Any other specialized examinations were conducted mainly at the biochemistry laboratories of either Robert Debre Hospital in Paris or Henri Mondor Hospital in Creteil (France). RESULTS: During the study period, 20 cases of a rare Hb were identified, i.e., 0.07% of all Hb analyses. These rare mutants were: HbK Woolwich (10 cases, 50% of the rare Hbs); HbD Korle-Bu (7 cases, 35%); HbJ Lome, G Philadelphia, and Hb Lepore (1 case each, 5% for each). Eighteen cases were associated with Hb A. Two cases of composite rare Hb were found: HbS/HbK Woolwich and HbC/Hb D Korle-Bu. Carriers of Hb Lepore and of HbC/HbD Korle-Bu had hypochromic and microcytic anemia. HbK Woolwich was found in the Adja-Ewé and Tem-Kabyé ethnic groups, in the Maritime and Kara regions; HbD Korle-Bu, HbJ Lome, and Hb Lepore were also identified among the Adja-Ewé, and HbG Philadelphia in the Akposso ethnic group of the Plateau zone. CONCLUSION: This study although limited to Lome, allowed us to identify the rare Hb forms observed in Togo. Other studies on a national scale will specify their real frequency.

[Chronic myeloid leukemia and imatinib: Experience at the Lome Campus teaching hospital (Togo)]. / Leucémie myéloïde chronique et imatinib, expérience du CHU Campus de Lomé au Togo.

BACKGROUND: Chronic myeloid leukemia (CML) is a clonal malignant myeloproliferative disorder characterized by the expansion of hematopoietic cells carrying the Philadelphia chromosome (t 9.22). Our main objective was to assess the efficacy of imatinib in CML patients, measured by their survival. METHODS: Over a six-year period (June 2003 through May 2009), 25 patients were seen regularly for CML at the Lomé Campus teaching hospital. Patients received imatinib after diagnosis and underwent regular laboratory monitoring (quantification of BCR-ABL ratio by RT-PCR). Patients' survival and treatment response were evaluated. RESULTS: Patients' mean age at diagnosis was 40 years (range: 9 to 72 years). Men predominated (17 compared with 7 women). Splenomegaly was found in 80% of cases. The mean leukocyte level was 188.71 g/L (24.4-350). Six patients (24%) had thrombocytosis with a mean platelet count of 491.15 g/L (108-2000). Six patients (24%) died after developing accelerated-phase CML or blast crisis. Estimated overall survival of patients at 6 years was 60%. Molecular biology monitoring detected a secondary G250E mutation with resistance to imatinib in one patient. Standard hematological side effects led to reduction in imatinib doses. The principal nonhematological side effects were weight gain and transient digestible disorders. CONCLUSIONS: At six years after diagnosis, imatinib was effective in treating patients with CML, even in sub-Saharan Africa. Mutation-induced resistance required regular molecular biological monitoring to determine the need to switch to later-generation tyrosine kinase inhibitors.

[Evaluation of the Widal-Felix serodiagnostic test in Togo]. / Evaluation de la pratique du sérodiagnostic de Widal et Félix au Togo.

PURPOSE: To evaluate the efficacy of Widal-Felix serodiagnostic testing in Togo. METHOD: This study using a cohort of 200 patients recruited at hospitals in Lome and Atakpame from November 2005 to April 2006 was designed to compare the sensitivity and specificity of the routinely used technique (plate agglutination) and reference technique (tube agglutination). RESULTS: Findings showed that the prevalence of typhoid fever was 1.5% while that of paratyphoid fever was nil. The hardest-hit age group was young people between 11 to 20 years. Men were two times more likely than women to be infected (sex-ratio, 0.5). The most useful symptoms for discriminating patients seropositive for Salmonella typhi from other serological profiles were abdominal pain (p<0.034) and diarrhea (p<0.008). The thick-drop malaria test was positive in all 3 patients (100%) with proven typhoid fever in comparison with 3.9% of the 154 patients with negative Widal-Felix serodiagnostic tests and 26.2% of the 42 patients with intermediate serological profiles. The sensitivity and specificity of the plate technique were 60.0% and 98.06% respectively. The sensitivity and specificity of laboratory analysis were 60.0% and 91.08% respectively. CONCLUSION: The gap between the specificity of the routine plate technique and laboratory analysis underscores the difficulty of performing and interpreting the Widal-Felix serodiagnostic test. The low sensitivity and specificity of the Widal-Felix serodiagnostic test also cast doubt on systematic use for diagnosis in patients presenting fever and on initiation of antibiotic treatment based on agglutination of a single antigen. To improve the specificity of the Widal-Felix serodiagnostic test, we recommend standardization of interpretation criteria and use of tube agglutination. We also see the need for development of another reproducible immunologic test for the diagnosis of typhoid and paratyphoid infections.

[Stuttering priapism in children with sickle cell anemia in Togo]. / Priapisme intermittent chronique chez l'enfant drépanocytaire homozygote au Togo.

AIM: To describe clinical aspects and outcome of stuttering priapism (SP) in children with sickle cell anemia. PATIENTS AND METHODS: We included the 8 children with sickle cell anemia suffering from SP between 1996 and 2004. RESULTS: Age at onset of SP ranged from 5 to 14 years (mean 9.75+/-3.69 years). SP attacks occurred once a day for 6 patients, and 2 and 3 times by week for the others. It happened during deep sleep, especially in the night (all patients) and sometimes in the day (4 patients). The duration of attacks ranged from 10 min to 2 h in 6 patients and 4 to 5 h in 2 patients. Oral étilefrine was administered to all patients until the cessation of attacks, and continued during 1 month. A long remission was obtained in all patients after 7 days to 7 months treatment (follow-up 2 to 7 years) in spite of 1 or many relapses.