Nonreplacement therapy for hemophilia in low-income countries: experience from a prospective study in Ivory Coast.

Background: Hemophilia management has fundamentally evolved over the last decades with the development of ground-breaking therapies. Because of their mode of action and biochemical properties, these innovative therapies that are available in developed countries could be readily implemented among people from low-income countries who are either not or inadequately treated with clotting factor concentrates (CFCs). Objectives: We aimed at evaluating the impact of prophylaxis with emicizumab, a bispecific monoclonal antibody mimicking the FVIII activity administered subcutaneously, among boys with severe hemophilia A (HA) from the Ivory Coast, where access to CFCs is limited to humanitarian aid. Methods: We prospectively collected data on the implementation and outcomes of prophylaxis with emicizumab, in 33 Ivorian boys aged 2 to 13 years with severe HA (with and without inhibitors). Bleeds, CFC consumption, quality of life and satisfaction of the patients and their parents were assessed. Results: Overall, 12 months after initiating emicizumab, a 99% reduction in bleeding rates was observed, with a raise from 18% to 100% of boys having zero spontaneous joint bleeds. Three boys required a single FVIII infusion following a traumatic bleed. Health-related quality of life measures significantly improved, and perception of treatment efficacy was positively rated in children and parents. Acceptance, tolerance, and adherence were excellent. Emicizumab was instrumental in successfully implementing uninterrupted, highly efficacious, and well-tolerated prophylaxis in 72% of the Ivorian children aged ≤ 13 years identified with severe hemophilia A. Conclusion: These data illustrate how innovative and disruptive nonreplacement therapies that are already accessible in developed countries could potentially provide equity in care by profoundly and rapidly modifying hemophilia burden with a magnified impact in low-income countries.

Feasibility and outcomes of low-dose and low-frequency prophylaxis with recombinant extended half-life products (Fc-rFVIII and Fc-rFIX) in Ivorian children with hemophilia: Two-year experience in the setting of World Federation of Haemophilia humanitarian aid programme.

INTRODUCTION: In sub-Saharan Africa, access to clotting factor concentrates (CFCs) is often extremely limited and published data on people with haemophilia on prophylaxis are almost not existent. AIMS AND METHODS: To assess the feasibility, barriers and outcomes of a low-dose and low-frequency prophylaxis with extended half-life (EHL) recombinant Fc fusion FVIII and FIX in Ivorian children on a two-year period in the setting of the World Federation of Hemophilia's (WFH) humanitarian aid programme. RESULTS: Twenty-five boys with haemophilia were included. Mean (SD) age at inclusion was 5.6 (2.5) years. The median [range] follow-up duration was 17 [11-24] months. Regimen of prophylaxis was 20 IU kg-1 1×/week in haemophilia A and every 10 days in haemophilia B. We observed a maximal reduction by 87.6% of the annual spontaneous joint bleeding rate and a slight decrease in the total HJHS scores (p = .047). Adherence problems related to parents' low education level and shortage in CFCs were the main issues to carry out the programme. Inhibitors occurred in 12.5%. CONCLUSION: This study confirms the feasibility and efficacy of low-dose and low-frequency prophylaxis in young Ivorian children with haemophilia treated with EHL CFCs donated through the WFH humanitarian aid programme. This work also highlights the crucial role of adherence and the need for appropriate education to achieve prophylaxis. Finally, it reminds the paramount objective of achieving self-sufficient, sustainable and available haemophilia replacement therapy for all worldwide.

Cross-cultural adaptation and validation of Haem-A-QoL in Côte d'Ivoire.

INTRODUCTION: Health-related quality (HRQoL) evaluations are considered essential outcomes in the assessment of people with haemophilia. In developing countries, reliable HRQoL data are even more critical whilst enabling government agencies to develop national haemophilia care programmes. However, validated tools are not yet available in sub-Saharan African countries. AIMS: This study sought to perform a cultural adaptation and validation of the Haemophilia Quality of Life Questionnaire for Adults (Haem-A-QoL) in Côte d'Ivoire. METHODS: The process comprised several steps, such as linguistic adaptation, cognitive debriefing interviews with adult haemophilia patients and psychometric testing, including reliability (internal consistency, test-retest reliability) and validity assessments (convergent with EQ-5D-5L, criterion with HJHS 2.1, known-groups). RESULTS: The final Ivoirian Haem-A-QoL version was obtained in December 2017 following linguistic adaptation and cognitive debriefings with six participants. The validation process included 25 patients, mainly haemophilia A patients (88%) with severe forms (80%). All participants received on-demand treatment, with joint impairment observed in 92%. Internal consistency and test-retest reliability of the Ivoirian Haem-A-QoL were very good. A Pearson correlation analysis revealed a moderate negative correlation between EQ-VAS and total Haem-A-QoL scores and a moderate positive correlation between HJHS 2.1 and total Haem-A-QoL scores. CONCLUSIONS: A cross-culturally adapted and validated Haem-A-QoL version in Côte d'Ivoire is now available, enabling measurement of intervention outcomes in the targeted population and Ivorian participation to multisite international trials. However, further work is needed to ensure optimal understanding of HRQoL questionnaires, previously developed in culturally distinct countries, with almost unlimited access to different treatment regimens.

Cross-cultural adaptation and validation of the Canadian Haemophilia Outcomes-Kids' Life Assessment Tool (CHO-KLAT) in Côte d'Ivoire (the Ivory Coast).

INTRODUCTION: Health-related quality of life evaluation is recognized as an important outcome in the assessment of boys with haemophilia. In fact, reliable health-related quality of life data are even more critical in developing countries to advocate for government agencies to develop national haemophilia care programmes. However, validated tools are not yet available in sub-Saharan African countries. AIMS: The purpose of this study was to complete the cultural adaptation and validation of the Canadian Haemophilia Outcomes-Kids' Life Assessment Tool version2.0 (CHO-KLAT2.0) in Côte d'Ivoire. METHODS: The process included four steps: a linguistic adaptation, cognitive debriefing interviews with children and their parents, a validity assessment with the Pediatric Quality of Life Inventory (PedsQL) as a comparator, and a test-retest reliability assessment. RESULTS: The initial Ivoirian version of the CHO-KLAT2.0 was developed through a linguistic adaptation performed in close collaboration with members of the local medical team and haemophilia community. Cognitive debriefings were completed with five boys and their parents, with the final Ivoirian version of the CHO-KLAT2.0 developed in September 2017. The validation process included 37 boys with haemophilia (mean age: 11.4 years; 34 with severe and three with moderate forms of haemophilia, all treated on demand) and their parents. Among the child-reported population (n = 20), we observed a mean CHO-KLAT2.0 score of 51.3 ± 9.2; there was a moderate correlation between the CHO-KLAT2.0 and PedsQL scores (r = 0.581; p = 0.007) and an inverse correlation of the CHO-KLAT2.0 and PedsQL scores with the global rating of the degree to which the boys were bothered by their haemophilia. The mean parent proxy CHO-KLAT2.0 score (n = 17) was 53.5 ± 9.8. Among the parents, we found no significant correlation between the Ivoirian CHO-KLAT2.0 and PedsQL scores or between the parent-reported scores and the parent global ratings of bother. The test-retest intraclass correlation coefficient was 0.879 (95% CI: 0.673; 0.954) for the child-reported questionnaires and 0.880 (95% CI: 0.694; 0.955) for the proxy-reported questionnaires. CONCLUSIONS: A cross-culturally adapted and validated version of the CHO-KLAT2.0 for Côte d'Ivoire is now available that enables baseline values to be obtained and intervention outcomes (namely, prophylaxis) to be measured in Ivoirian boys with haemophilia.

Inhibitor epidemiology and genetic-related risk factors in people with haemophilia from Côte d'Ivoire.

INTRODUCTION: In Sub-Saharan Africa, inhibitor prevalence data in people with haemophilia (PWH) are scarce, as are data on genetic or treatment-related risk factors. AIMS AND METHODS: We performed a prospective study on PWH from Côte d'Ivoire to collect data into inhibitor prevalence, create a database of haemophilia genotypes, establish correlations between inhibitor presence and genetic variants identified amongst Ivoirian PWHs and evaluate exposure to CFCs. RESULTS: The study included 54 unrelated participants (43 severe, four moderate, two mild haemophilia A and five severe haemophilia B). PWH were treated on-demand with various product types for short periods, non-intensively, and using low-dose regimens. We reported similar distributions of intron 22 inversions (39.5%), point pathogenic variants (32.6%) and rearrangements in Ivoirian severe haemophilia A patients versus non-African ethnic groups. The haplotypes H1 (29.6%), H2 (36.3%) and H3 (34.1%) frequencies in haemophilia A were consistent with results published on African populations. We identified eight new causal variants. An inhibitor was found in 12% of haemophilia A patients previously exposed to replacement therapies. Among PWH with inhibitors, 66.7% had a positive intron 22 inversion and 50% the H1 haplotype. CONCLUSION: This study provides original data on molecular diagnosis of haemophilia, inhibitor prevalence and risk factors for inhibitor development previously associated with inhibitors in Côte d'Ivoire. The low inhibitor prevalence likely reflects the limited exposure to replacement therapy in Côte d'Ivoire. Further larger, multicentric and international studies are needed to gain more insight on inhibitor incidence and risk factors in African PWH.

Implementation and assessment of a self- and community-based rehabilitation programme in patients with haemophilia from Côte d'Ivoire.

INTRODUCTION: In resource-constrained countries, few patients with haemophilia (PWH) have access to clotting factor concentrates (CFC), with increased musculoskeletal (MSK) complications. Physiotherapy actively contributes to preventing MSK complications, minimizing joint damage and reducing pain. AIM: To assess the impact of a 20-week self- and community-based rehabilitation (CBR) programme in Ivorian PWH. METHODS: Fifty participants underwent a clinical and functional baseline assessment with identification of joints' functional defects and initiation of an individualized exercise programme comprising exercises to improve strength, joint mobility and proprioception. Hemophilia Joint Health Score (HJHS), 2-minute walking test (2MWT), Timed Up and Go (TUG), goniometry and maximal isometric voluntary contractions using the MicroFET2 were performed at baseline (T1) and at Week 20 (T2). RESULTS: At T2, there was a significant improvement in both the 2MWT and TUG tests. The HJHS total score decreased significantly from 23.6 ± 14.2 at T1 to 20.4 ± 13 at T2. A significant improvement in joint health was found in the left elbow, right knee and right ankle, with elements correlating with joint function responsible for these improvements. A strong programme adherence was observed, with 94% of participants reporting regular exercise performance and a high degree of satisfaction. CONCLUSION: The programme with its encouraging results is meant to be the first step towards a more ambitious project. Self-based and CBR programmes are inexpensive and efficient treatment options designed to minimize the detrimental effects of joint and muscle bleedings, and to increase the functional independence and quality of life of PWH with limited access to CFC and physiotherapy.

Development and evaluation of appropriate, culturally adapted educational tools for Ivoirian patients with haemophilia, haemophilia carriers and their families.

INTRODUCTION: Patient education is the cornerstone of the management of chronic diseases like haemophilia. The education of patients with haemophilia (PWH), haemophilia carriers and their families requires educational materials adapted to their socio-cultural situations for maximum effectiveness. These tools are currently lacking in developing countries like Côte d'Ivoire. AIMS: We sought to develop educational materials adapted to the Ivoirian context, assess their short- and long-term impacts on knowledge about haemophilia and evaluate the participants' motivation and their satisfaction with the tools. METHODS: Following their elaboration, the materials were administered to 71 participants (37 PWH, 29 carriers and 5 fathers), whose level of knowledge was assessed before (T0), just after (T1), and 1 year following the intervention (T2). We evaluated, analysed and compared the scores at T0, T1 and T2 and evaluated motivation at T0 and satisfaction at T1. RESULTS: All participants significantly improved their skills at T1 (P < 0.001), maintaining a sustained and significant improvement at T2 in comparison with T0 (P < 0.001). In all participants, we observed a high degree of motivation towards improving their knowledge and a high degree of satisfaction with the materials. CONCLUSIONS: Appropriate, culturally adapted educational tools focused on haemophilia are now available in Côte d'Ivoire. These materials will likely contribute to improving haemophilia awareness, to implementing screening, prevention and self-management of the disease and to positively impacting the outcomes of Ivoirian PWH in the long term.

[Pre-therapeutic and evolutive characteristics of patients suffering from chronic myeloid leukemia, in Abidjan, Ivory Coast]. / Caractéristiques préthérapeutiques et évolutives des patients atteints de la leucémie myéloïde chronique à Abidjan Côte d'Ivoire.

INTRODUCTION: The diagnosis of chronic myeloid leukemia is based on the presence of translocation t(9,22). Additional cytogenetic abnormalities may exist at diagnosis and have prognostic value. The authors evaluated the relationship between these additional chromosomal abnormalities, clinical presentation, and therapeutic response. METHOD: In a retrospective and comparative study from 2005 to 2015, at Yopougon university hospital, 51 cases of myeloid leukemia were selected, including 22 cases with additional chromosomal abnormalities. RESULTS: Thirteen types of additional Ph1 abnormalities were detected in one group, with a median age of 39years (13-73); a sex ratio of 1.4 and a low social class (49%). The median consultation time is 13months (2-29). Hepatomegaly (54%, P=0.05); fever (81.8%, P=0.0017); bone pain (63.6%, P=0.0001); lymphadenopathies (27.3% P=0.014); poor general condition [WHO>1 (77.3%, P=0.001)], high Sokal index (63.6%, P=0.0019), eosinophilia>5% (72.7, P=0.02) and circulating blastosis were found more frequent in the group with additional abnormalities treated with imatinib mesylate. We obtained 13.6% hematologic remission and 22.7% cytogenetic remission (P=0.02). The average survival was relatively short (20months vs. 76.4months, Log-rank<0.0001). We deplored a high death rate (59.1%). CONCLUSION: The presence of an additional anomaly constitutes a pejorative element refractory to imatinib.

Haemophilia in Côte d'Ivoire (the Ivory Coast) in 2017: Extensive data collection as part of the World Federation of Hemophilia's twinning programme.

INTRODUCTION: In sub-Saharan African countries, research on haemophilia is limited. Since 2015, a partnership has been established through the World Federation of Hemophilia (WFH)'s twinning programme between the haemophilia treatment centre (HTC) of the Centre Hospitalier universitaire of Yopougon in Abidjan, Côte d'Ivoire, and the Cliniques universitaires Saint-Luc of Brussels, Belgium. AIM: This study sought to collect accurate, and detailed demographic, clinical, and laboratory data on the whole identified Ivorian haemophilia population. METHODS: A prospective study was conducted in 2017 in Yopougon's HTC. Participants were assessed through multidisciplinary workups including interviews, logbook review, pedigree establishment, clinical examination and laboratory testing. RESULTS: Data on 81 patients with haemophilia (PWH) (78 severe and moderate) were collected. Postcircumcision bleeding was the most common diagnosis reason (32%). Mouth bleeds and skin wounds accounted for 55.2% of bleeds. Pedigrees revealed 63 deaths in affected relatives among 33 families. Most PWHs (76.5%) were treated on demand, and 21% had never been exposed to clotting factor. Non-substitutive therapies (tranexamic acid [43%], physiotherapy [11%] and DDAVP [0%]) were underused. Overweight was uncommon. Knees were the most clinically affected joints at the Hemophilia Joint Health Score. Inhibitors were present in 7.8% of previously treated PWHs. CONCLUSIONS: This study highlights the value of simple, feasible and inexpensive tools to collect data in the Ivorian haemophilia population and provides the basis for developing and implementing locally appropriate strategies to improve screening, diagnosis, preventive care, treatment and education. It demonstrated the WFH twinning programme benefits for haemophilia care in the developing world.

Hemophilia carrier's awareness, diagnosis, and management in emerging countries: a cross-sectional study in Côte d'Ivoire (Ivory Coast).

BACKGROUND: Little data is available on awareness of hemophilia carrier condition or associated bleeding risk and management in Sub-Saharan African countries. This study sought to identify hemophilia carriers in Côte d'Ivoire in order to collect data on demographics, bleeding phenotype, and laboratory results. Another purpose was to provide Ivorian hemophilia carriers with counseling on their risk of bleeding and of having children with hemophilia. A 12-month prospective study was conducted involving Ivorian hemophilia carriers recruited trough pedigree analysis pertaining to 81 hemophilia patients followed-up at the Yopougon Hemophilia Treatment Center in Abidjan. They were assessed using in-depth interviews, pedigree analysis, and laboratory testing. RESULTS: Sixty-one subjects comprising 27 obligate and 34 possible carriers were recruited. None had previously been assessed, with 64% unaware of their carrier status despite a familial history of hemophilia in 69%. The most frequently reported bleeding symptom was menorrhagia (31%). Prolonged bleeding was reported after vaginal delivery in 19.6%, post-surgery in 4.9%, and post-dental extraction in 4.9%. Only one carrier was treated with tranexamic acid, with no other hemostatic therapy recorded. The median (range) clotting FVIII was 0.85 IU/mL (0.24-1.90 IU/mL) and FIX 0.60 IU/mL (0.42-1.76 IU/mL) in hemophilia A and B carriers, respectively. HA carriers had a FVIII < 0.5 IU/mL in 12.5%. CONCLUSIONS: This study highlights the need of implementing care for hemophilia carriers in developing countries, and the high value of pedigree analysis for carrier identification, along with the relevance of diagnosis, treatment, and education of carriers, families, and caregivers.

[Retrospective study about 20 cases of ovarian Burkitt lymphoma at Yopougon teaching hospital in Côte d'Ivoire]. / Étude rétrospective rapportant 20 cas de lymphome de Burkitt de localisation ovarienne pris en charge au CHU de Yopougon en Côte d'Ivoire.

CONTEXT: The maxillo-facial attack was the first described in the Burkitt lymphoma in 1958 by Denis Pearsons Burkitt. Abdominopelvic disorders, particularly ovarian localization are observed more and more by the developments of imagery technics. Our study aimed to describe the epidemiologic, clinical, therapeutic and evolutive aspects of ovarian localization in the endemic Burkitt lymphoma. METHODOLOGY: Twenty files of ovarian Burkitt lymphoma diagnosed in the clinical service of hematology TH of Yopougon during the period August 1995-March 2015 (19 years) were retrospectively analyzed. The epidemiologic, clinical, therapeutic and evolutive parameters were studied. RESULTS: Ovarian Burkitt lymphoma accounted for 20.41% of the population with Burkitt lymphoma and 38.46% for patients with Burkitt lymphoma. The average age was 20.4 years with extremes of 6 and 38 years. The main reasons for consultation were general impairment (85%) and pelvic mass (80%). 75% were in group B clinical scalability. The ovarian mass was bilateral in 60% of cases, heterogeneous in 75% of cases. There was a clear predominance of stage III of Murphy (55%). On the evolutionary level all the patients treated by chemotherapy were in incomplete remission (100%). 10% were alive. 83.33% of the deceased patients had received less than 6 courses of chemotherapy. CONCLUSION: The diagnosis of ovarian endemic Burkitt lymphoma is most often delayed diagnosis and poor prognosis. Improving its management requires early diagnosis.

Les atteintes osseuses drepanocytaires

Contexte : Les complications osseuses chez le drepanocytaire telles que l'osteonecrose; l'osteoarthrite et l'osteomyelite constituent une grande source de morbidite compromettant le plus souvent le pronostic fonctionnel du membre du patient. Patients et Methode : Il s'agissait d'une etude retrospective; descriptive realisee sur sur une periode 10 ans et concernant 210 patients drepanocytaires ayant presente au moins une des trois complications osseuses majeures. Resultats : L'age moyen etait de 19 ans avec des extremes de 02 a 43 ans. On notait un sex-ratio de 1;1 en faveur des hommes. Les atteintes osseuses sont l'apanage des formes homozygotes SSFA 2 (42;9) et ? 0 thalasso-drepanocytaires SFA 2 (26;2). Il s'agissait essentiellement d'osteomyelite (43;3); osteonecrose (47;2) et osteo-arthrite (9;5). Les localisations preferentielles de l'osteomyelite etaient le tibia et le perone (12;8); le femur (7;1) et l'humerus (6;2). La tete femorale (45;7) et humerale (1;4) etaient deux localisations quasi-constantes de l'osteonecrose. Quant a l'osteo-arthrite; l'atteinte de la hanche etait estimee a 24 des cas et le genou 5;7

Resultat therapeutique et survie des lymphomes malins B diffus a grandes cellules dans le service d'hematologie clinique du CHU de Yopougon

"Objectif : Etudier la survie des patients atteints de lymphomes malins B diffus a grandes cellules. Population et methodes : Il s'agissait d'une etude retrospective; descriptive; analytique et non comparative portant sur 65 dossiers de patients atteints de lymphome B diffus a grandes cellules suivis dans le service d'hematologie clinique du CHU de Yopougon sur une periode de 20 ans. La survie a ete etudie selon la methode de Kaplan-Meir et la comparaison des courbes de survies selon le test de"" Log Rank"" Resultats : la moyenne d'age etait de 42 ans. Le sexe masculin predominait avec un sex-ratio de 1;9. La prise en charge d'un lymphome malin non Hodgkinien etait le motif de consultation le plus frequent dans 85%. 44 patients avaient un PS egal a 2 et la localisation ganglionnaire primitive etait majoritaire dans 69;2%. 86% presentaient des signes d'evolutivites cliniques; le type histologique centroblastique predominait (65%). 4 malades presentaient une serologie VIH positive sur 14 serologies demandees. Le stade d'ANN ARBOR III et IV etaient majoritaires (63% ). 83% des patients presentaient des signes d'evolutivites biologiques. Les taux de la lacticodeshydrogenase et de la ?2 microglobuline etaient eleves dans respectivement 83% et 86%. Le taux d'hemoglobine etait comprit entre 7g /dl et 12g/dl dans 77%. Le protocole CHOP etait le plus utilise dans 56%; la reponse therapeutique a ete evaluee chez 26 patients avec un taux de remission complete(RC) de 65%; la duree de la RC etait superieure a un an dans 47.06%. 91% de nos malades sont decedes. La survie globale a 1 an etait de 40% et a 5 ans de 5% avec une survie moyenne de 11 mois. Aucun des parametres sociodemographiques; cliniques; biologiques et therapeutiques analyses n'avait influence significativement la survie. "

Imatinib Mesylate Effectiveness in Chronic Myeloid Leukemia with Additional Cytogenetic Abnormalities at Diagnosis among Black Africans.

Imatinib mesylate provides good results in the treatment of CML in general. But what about the results of this treatment in CML associated with additional cytogenetic abnormalities at diagnosis among black Africans? For this, we retrospectively studied 27 cases of CML associated with additional cytogenetic abnormalities, diagnosed in the department of clinical hematology of the University Hospital of Yopougon in Côte d'Ivoire, from May 2005 to October 2011. The age of patients ranged from 13 to 68 years, with a mean age of 38 years and a sex ratio of 2. Patients were severely symptomatic with a high Sokal score of 67%. CML in chronic phase accounted for 67%. The prevalence of additional cytogenetic abnormalities was 29.7%. There were variants of the Philadelphia chromosome (18.5%), trisomy 8 (14.8%), complex cytogenetic abnormalities (18.5%), second Philadelphia chromosome (14.8%), and minor cytogenetic abnormalities (44.4%). Complete hematologic remission was achieved in 59%, with 52% of major cytogenetic remission. The outcome was fatal in 37% of patients. Death was related in 40% to hematologic toxicity and in 30% to acutisation. The median survival was 40 months.

Characteristics and Results of the Treatment of Multiple Myeloma in the Subject under the Age of 65 at the University Hospital of Yopougon in Abidjan, Côte d'Ivoire.

We retrospectively studied 30 cases of multiple myeloma in patients under the age of 65, diagnosed from 1991 to 2005 in the clinical hematology department of the University Hospital of Yopougon that is a hospital incidence of 2.9 cases/year. The age of patients ranged from 34 to 64 years, with a mean age of 49 years and a sex ratio of 1.73. The professional activity was variable with 3% of radiographers and 10% of farmers. Clinically, the dominant sign was bone pain in 83% of cases. Myeloma was secretory in 93% of cases. It was Ig G-type in 86%, kappa-type in 66% of cases. 86% of patients were anemic, 20% had creatinine >20 mg/L, and 10% had serum calcium >120 mg/L. Geodes were found in 80% of cases. 53% were at stage III of DURIE and SALMON. Complications were infectious (33%), renal (20%), and hemorrhagic (7%). Chemotherapy regimens were VAD (10%), VMCP (30%), and VMCP/VBAP (60%) with 47% of partial responses, 33% of stable disease, and 7% of very good quality partial responses. The outcome developed towards death in 37% and causes of death were renal in 46% of cases. The median survival was only 5.1 months.

Characteristics and Results of the Management of Diffuse Large B-Cell Lymphomas: The Experience of Côte d'Ivoire.

Diffuse large B-cell lymphomas have been little studied in black Africans. The purpose of our study was to determine the characteristics and results of the management of these lymphomas. Patients and Methods. In a descriptive and analytic retrospective study we studied the medical records of 63 patients with diffuse large B-cell lymphoma hospitalized during the period from 1991 to 2007. The diagnosis was made after lymph node or organ biopsy. Response to treatment, OS, PFS, and toxicity were studied. The complete response has been analyzed univariate and multivariate analysis. Results. The median age was 42 years. The sex ratio was 2. The HIV serology was positive in 11 cases, and 8 patients had antiretroviral therapy. In 71% the lymphoma was at stages III and IV of Ann Arbor. IPI was ≥3 in 65%. Complete remission was achieved in 43%. Only 43% of patients had had a good compliance. Progression-free survival at 3 years was 32%, and overall survival at 3 years was 50%. 13% of patients were lost to follow up, and 51% of them died. In terms of analysis the complete remission rate was influenced by the stage of Ann Arbor (P < 0.0001), biological b symptoms (P < 0.01), the IPI (P < 0.0001), and the socioeconomic standing (P = 0.001). In multivariate analysis, only IPI and stage of Ann Arbor influence the complete remission.

The Effect of Imatinib Mesylate for Newly Diagnosed Philadelphia Chromosome-Positive, Chronic-Phase Myeloid Leukemia in Sub-Saharan African Patients: The Experience of Côte d'Ivoire.

Imatinib mesylate, showed encouraging activity in chronic myelogenous leukemia. However, there are few data regarding his efficacy and response monitoring in Sub-Saharan African patients. Our objective was to assess response to imatinib mesylate (Glivec) in Côte d'Ivoire patients with newly diagnosed Chronic Myeloid Leukemia (CML). From May 2005 to September 2009, we treated 42 patients (40 years; range 16-69) with Philadelphia chromosome (Ph+) positive in chronic phase CML with oral imatinib mesylate at daily doses of 400 mg. Overall survival (OS) and frequency of complete or major cytogenetic remission (CCR/MCR) were evaluated. At a median follow up of 32 (range 7.6-113) months, the CHR rate in our study group was 76%. A major CR was found in 19 patients (45%) with 17% and 29% complete and partial CR respectively. There were no significant differences in the incidence of major cytogenetic response by known prognostics factors. Median time to CHR was 8 months (range 0.4-25), and 16 months (range: 0.1-36) for CR. Projected 5-year OS rate was 72% (95%CI 42-88). We conclude that imatinib therapy sub-Saharan African CML patients is very promising and has favorably changed the prognosis for black African patients with CML.

[Rare localisations of endemic Burkitt lymphoma: about 21 cases observed in the Haematology Department of the University Hospital of Yopougon Abidjan]. / Localisations exceptionnelles du Lymphome endémique de Burkitt (à propos de 21 cas vus en Côte d'Ivoire).

In this retrospective study, we analysed rare localisations of Burkitt lymphoma observed in the Haematology Department of the University Hospital of Yopougon Abidjan. Over a 10-year study period, we saw 106 patients with Burkitt lymphoma, 21 with unusual localisations. The mean age at onset of symptoms or discovery was 15.48 years, and more patients were female. There were 8 cases of primary Burkitt lymphoma in a rare localisation and 13 cases of unusual localisations of secondary lymphomas. The most frequent of these rare lesions were renal (6), ovarian (4), in peripheral nodes (4), testicular (3), and mammary (3). Still rarer were thyroid, skin, pulmonary, and suprarenal localisations. Clinical manifestations were non-specific and difficult to differentiate from other solid tumours. The CMA protocol produced complete remission in 29% of cases (n=6). Seven patients died. In conclusion, the rare forms of Burkitt lymphoma are non-specific and can be mistaken for other solid tumours. Diagnosis should thus be based on histochemical analysis.

[Homozygous sickle cell disease in Ivory Coast adults]. / Drépanocytose homozygote chez l'adulte ivoirien de plus de 21 ans.

Sickle cell disease is a genetic disease characterized by the synthesis of an abnormal haemoglobin called haemoglobin S. It is the most frequent of the hereditary anomalies of haemoglobin and occurs most commonly in individuals of African descent. Various treatments have considerably improved its prognosis, prolonging the survival of patients, especially those with the most severe, homozygous form. The objective of thisstudy is to describe the epidemiologic, clinical, and laboratory characteristics as well as the disease course and available treatments in adults (aged 21 years or older). This retrospective, descriptive, analytic and non-comparative study included 48 adults of both sexes with homozygous sickle cell disease. Their mean age was 26.1 years (range: 21 to 56 years, and sex ratio 1.3. In all, 70.8% had clinical anaemia, 83.3% were subicteric or icteric and 8.3% had hepatomegaly. Spleen size was normal in 41.7% of patients, and atrophic in 37.5%. No case of splenomegaly was noted and 8.3% had been splenectomised. Haemoglobin rates ranged from 4 g/dL to 12.7 g/dL with an average of 9.5 g/dL, haemoglobin S levels from 83 to 93% with an average of 85.3%, and haemoglobin F levels from 3.5 to 17% with an average of 10.6%. The percentage with fewer than three crises (vasooclusive or haemolytic or both) in a year was 68.7%; 27.1% had from three to five crises, and 4.2% more than five. Disease complications included anaemia in 43.7%, infections in 18.8% and ischaemia in 16.7%; 20.8% had no complications. Age at the beginning of treatment was younger than 5 years in 56.25%, from 5 to 10 years in 29.2%, and older than 10 years in 14.6%. Medical follow-up was regular for 68.7% and irregular for 31.2%. Vaccination was up to date in 58.3. Most patients (83.3%) adhered to their maintenance treatment. In all, 41.7% had not had any blood transfusions, 54.2% had had one or two transfusions, and 4.2% three or more. We compared the patients aged 26 years or younger with those older than 26 and studied the influence of age on different disease variables. Age did not affect the frequency of crises (p = 0.368) or of infections (p = 0.116), the rates of haemoglobin (p = 0.221), haemoglobin S (p = 0.44), or haemoglobin F (p = 0.35), or complications (p = 0.56). Nevertheless, we noted that the frequency of crises, infections, and anaemic complications were higher among the younger patients. Early treatment, regular medical follow-up, maintenance treatment and vaccination have all improved the prognosis of homozygous sickle cell disease considerably. These patients have reached adulthood with relatively few chronic complications.

Results of treatment with CMA, a low intermediate regimen, in endemic Burkitt lymphomas in sub-Saharian Africa: experience of Côte d'Ivoire.

African Burkitt lymphomas (BL) are highly aggressive lymphomas mainly affecting children and young adults in Africa. This lymphoma was marked by its high sensitivity to chemotherapy in comparison to Sporadic Burkitt lymphoma. In this study, we evaluated the treatment response and survival of patients with CMA protocol. Eighty-five of the 105 children registered were evaluated for response; there were 46 boys and 39 girls, whose age at diagnosis ranged from 3 to 18 years (median 11 years), admitted to the Hematology National Teaching Hospital of Abidjan in the period 1998-2008 with a diagnosis of BL on histological review and who were given CMA chemotherapy with curative intent are included in this analysis. CMA protocol is a low intermediate regimen of 3 drugs [Cytarabin (ara-C), Methotrexate (MTX), and Cyclophosphamide] with CNS-directed treatment by intrathecal MTX, ara-C and corticosteroid. Fifty-five of 85 patients obtained CR after induction therapy and 10 after 3 supplementary cycle because of partial response. The overall complete remission was 76%. Fifty-three of patients were alive in first CR at a median survival rate period of 2 years (range 82 days to 9 years) and are continuously disease free from Burkitt lymphomas. Twelve patients relapse after CR and died of lymphoma progression. More than 32 patients died, as a result of lymphoma progression. Among the 32 dead, 10 were in Murphy stage IV and all the patients who presented bone marrow involvement died. The projected 5-year overall survival rate was 62%. In conclusion, CMA protocol shows the high sensitivity of African Burkitt lymphoma. This can be considered as a successful result for people living in poor socio-economic conditions with no health insurance.