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.

[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.