Incidence et caractéristiques des hémopathies malignes au cours de l’artérite à cellules géantes

Introduction L’arterite a cellules geantes (ACG) est une vascularite du sujet âge au cours de laquelle les cellules mononucleees jouent un role physiopathologique majeur. L’hematopoiese clonale est un phenomene dont la frequence augmente egalement avec l’âge et qui conduit a l’apparition de mutations responsables de la survenue d’hemopathies malignes (HM) ou de mutations des cellules effectrices de l’immunite responsables de pathologies inflammatoires. Notre hypothese est qu’il existe une association specifique entre certaines HM et l’ACG. Nous avons pu etudier cette hypothese en croisant les donnees du registre des hemopathies de Cote d’Or (RHEMCO) et celles de tous les laboratoires d’anat…

Different profile and distribution of antigen specific T cells induced by intranasal and intrarectal immunization with rotavirus 2/6-VLP with and without LT-R192G

International audience; In this study, we compared both the profile and distribution of antigen specific primed T cells after intrarectal (IR) and intranasal (IN) immunization with rotavirus (RV) 2/6-VLP, alone or in the presence of LT-R192G, in order to highlight the differences between the two routes and the impact of the adjuvant. Adult BALB/c mice were immunized once with 2/6-VLP with or without adjuvant and the T cell response was analyzed in lymphoid tissues after in vitro restimulation with the antigen. IN, but not IR, immunization of mice with 2/6-VLP alone induced antigen-specific IL-10 and IL-17 secreting T cells. IL-10-, in contrast to IL-17-, secreting T cells did not migrate to…

Concordance of assays designed for the quantification of JAK2V617F: a multicenter study

Background Many different techniques have been designed for the quantification of JAK2 V617F allelic burden, sometimes producing discrepant results. Design and Methods JAK2 V617F quantification techniques were compared among 16 centers using 11 assays based on quantitative polymerase chain reaction (with mutation-specific primers or probes, or fluorescent resonance energy transfer/melting curve analysis), allele-specific polymerase chain reaction, conventional sequencing or pyrosequencing. Results A first series of blinded samples (granulocyte DNA, n=29) was analyzed. Seven assays (12 centers) reported values inside the mean±2SD; the mean coefficient of variation was 31%. Sequencing techniq…

Outcome of Refractory Anemia with Ringed Sideroblasts Associated with Marked Thrombocytosis (RARS-T) In a Large Cohort of Patients

Abstract Abstract 4113 Introduction: Most of the data related to RARS-T, a rare disorder, involve small cohorts of patients. We aimed to analyze more patients also considering a variety of myelodysplastic or myeloproliferative disorders. Objective: To compare a large cohort of patients with RARS-T to refractory anemia with ringed sideroblasts (RARS), refractory anemia with ringed sideroblasts and multilineage dysplasia (RARS-MD) or essential thrombocythemia (ET) at the time of diagnosis and during disease evolution, in terms of survival and complications. Materials: Data of a European multi-center study was used including 199 cases of RARS-T 173 cases of RARS, 102 cases of RARS-MD and 431 c…

The heterogeneity of changes in incidence and survival among lymphoid malignancies in a 30-year French population-based registry.

Our specialized population-based registry has allowed us to explore changes in incidence and survival by subtype over the last 30 years. Between 1980 and 2009, 4790 cases of lymphoid malignancies were registered using the International Classification of Diseases for Oncology. The incidence rate of lymphoid malignancies was 20.5 per 100,000 inhabitants per year, and ranged from 0.1 to 4 according to subtype. Five-year net survival was 65%, and ranged from 41% to 93% according to subtype. We observed an increase in 5-year net survival between the periods 1980-1989 and 2000-2009 (58% vs. 70%). This was observed in most but not all subtypes. Our long-standing population-based registry allowed u…

5-Year Overall Survival and Progression-Free Survival in Multiple Myeloma by Line of Treatment from the French Hematologic Malignancy Registry

Splenic Marginal Zone Lymphoma: French Registries Population-Based Treatment and Survival Analyses (2002-2014)

Background. Splenic Marginal Zone Lymphoma (SMZL) is a rare indolent B-cell lymphoma characterized by a massive splenomegaly and a moderate lymphocytosis. There is no standard of care for SMZL so far. The treatment is indicated if constitutional symptoms, massive splenomegaly and/or cytopenia, after a watch and wait period that is highly variable. Rituximab with or without chemotherapy, chemotherapy, and splenectomy are valid treatment approaches. Our objectives were to describe the characteristics of the patients with SMZL retrospectively collected from a French population database, to analyze the treatments received in first line (Tt1) and in second line (Tt2) in term of efficacy, and to …

Infradiaphragmatic Hodgkin lymphoma: a large series of patients staged with PET-CT

// Cedric Rossi 1, 2 , Morgane Mounier 3 , Pauline Brice 4 , Violaine Safar 5 , Emmanuelle Nicolas-Virelizier 6 , Philippe Rey 6 , Aspasia Stamatoullas-Bastard 7 , Marion Alcantara 7 , Adrien Chauchet 8 , Emilie Reboursiere 9 , Lauriane Filliatre 10 , Aurore Perrot 10 , Sylvain Garciaz 11 , Gilles Salles 6 , Bertrand Coiffier 6 , Herve Ghesquieres 5, 6 and Rene-Olivier Casasnovas 1, 12 1 Hematologie Clinique, CHU Le Bocage, Dijon, France 2 INSERM UMR 1037 - Cancer Research Center of Toulouse, Toulouse, France 3 Registre des Hemopathies Malignes de Cote d’Or, EA4184, Universite de Bourgogne, Dijon, France 4 Hematologie Clinique, CHU Paris-GH St-Louis Lariboisiere F-Widal - Hopital Saint-Loui…

Incidence and Prognostic Value of FLT-3 and NPM1 Genes Abnormalities in Acute Myeloid Leukemia in the Côte D’or Population, France

Abstract Context: In acute myeloid leukemia (AML), the recently described FLT-3 and NPM1 genes abnormalities were found to have a prognostic value in AML with normal karyotype and a specific therapeutic strategy was proposed according to these abnormalities. We look for the incidence and prognostic value of these abnormalities in cases diagnosed on a well defined population. Material and Methods: AML diagnosed according to WHO classification between 01/01/2001 and 31/12/2006 in the population of the Côte d’Or department, were included. Karyotype analyses were performed in 81% of the cases. The FLT3 D835 mutation, the FLT3 internal duplication (ITD) and the NPM1 mutation were systematically…

Trends in excess mortality in follicular lymphoma at a population level

Background Since the 1990s and since the development of humanised monoclonal antibodies in 1998, the treatment of non-Hodgkin lymphoma has undergone profound changes. Follicular lymphoma (FL) was the first to benefit from this treatment, and several clinical trials have shown a significant improvement in overall survival, but little information is available at a population level. Objective Our objective was to estimate changes in FL-specific mortality at a population level, with an appropriate methodology. Methods Two French retrospective population-based studies on FL were conducted, one from 1995 to 2004, in 1477 patients, and one from 1995 to 2010, in 451 patients. Trends in excess morta…

Evans’ Syndrome: From Diagnosis to Treatment

Evans’ syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. ES is a rare situation that represents up to 7% of AIHA and around 2% of ITP. When AIHA and ITP occurred concomitantly, the diagnosis procedure must rule out differential diagnoses such as thrombotic microangiopathies, anaemia due to bleedings complicating ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal haemoglobinuria, or specific conditions like HELLP when occurring during pregnancy. As for isolated auto-immune cytopenia (AIC), the determination of the primary or…

Les néoplasies plasmocytaires et autres dysglobulinémies en population générale

Resume Les neoplasies plasmocytaires occupent en 2012 en France le premier rang des hemopathies malignes en nombre. Majo-ritairement il s’agit de myelome multiple ou de plasmocytome (MM/P) mais on retrouve aussi des pathologies de severite variable. La classification OMS actuelle definit les gammapathies monoclonales de signification indeterminee en une entite a part entiere. Des progres importants dans la prise en charge de ces pathologies ont ete realises grâce a une meilleure connaissance de la maladie et a l’utilisation de nouvelles molecules therapeutiques. Ces progres peuvent etre visualises par l’etude des tendances d’indicateurs epidemiologiques en population generale. Les donnees f…

Increased Risk of Acute Myeloid Leukemias and Myelodysplastic Syndromes in Patients Who Received Thiopurine Treatment for Inflammatory Bowel Disease.

Background & Aims Treatment with immunosuppressive thiopurines such as azathioprine is associated with an increased risk of leukemogenesis. We assessed the risk of myeloid disorders, such as acute myeloid leukemia and myelodysplastic syndromes, in a large cohort of patients with inflammatory bowel disease (IBD) in France. Methods We performed a prospective observational study of 19,486 patients with IBD enrolled in the Cancers Et Surrisque Associe aux Maladies inflammatoires intestinales En France (CESAME) study from May 2004 through June 2005; patients were followed through December 31, 2007. The incidence of myeloid disorders in the general population, which was used for reference, was de…

Caractérisation de l’anémie hémolytique auto-immune associée aux hémopathies lymphoïdes à partir du registre des hémopathies malignes de Côte d’Or

Introduction L’anemie hemolytique auto-immune (AHAI) est une cytopenie auto-immune (CAI) caracterisee par une destruction des globules rouges par des auto-anticorps diriges contre certains de leurs antigenes de surface. Son incidence est estimee entre 1 et 3/100 000 personnes-annees (PA). Les AHAI a auto-anticorps chauds sont les plus frequentes et les formes secondaires, observees dans la moitie des cas, ont tendance a augmenter avec l’âge, notamment en association aux hemopathies malignes. Les donnees de la litterature reposent cependant sur de faibles cohortes ou des centres tertiaires, avec un possible biais de recrutement. L’objectif de notre etude est de decrire la frequence de l’AHAI…

Clinical Features And Course Of Refractory Anemia With Ring Sideroblasts Associated With Marked Thrombocytosis

Background Refractory anemia with ring sideroblasts associated with marked thrombocytosis was proposed as a provisional entity in the 2001 World Health Organization classification of myeloid neoplasms and also in the 2008 version, but its existence as a single entity is contested. We wish to define the clinical features of this rare myelodysplastic/myeloproliferative neoplasm and to compare its clinical outcome with that of refractory anemia with ring sideroblasts and essential thrombocythemia. Design and Methods We conducted a collaborative retrospective study across Europe. Our database included 200 patients diagnosed with refractory anemia with ring sideroblasts and marked thrombocytosis…

Impact de l’âge et de l’année sur les indicateurs de survie nette et de guérison par sous-type de leucémie aiguë myéloïde en France entre 1989 et 2010

Etat de la question Estimer la survie nette et les indicateurs guerison par sous-type de leucemie aigue myeloide (LAM) en France. Materiel et methodes Au total, 6460 cas de LAM dont l’âge ≥ 15 ans, diagnostiques entre 1989 et 2010 ont ete inclus. Cinq sous-types de LAM ont ete definis a l’aide de la classification ICD-O-3 et HAEMACARE. La survie nette a ete obtenue en utilisant le modele flexible ajuste sur l’âge, le sexe et l’annee de diagnostic pour chaque sous-type de LAM. Lorsque l’hypothese de guerison etait acceptable, un modele de guerison flexible a ete utilise pour estimer la proportion de gueri (P), le temps de guerison (TTC) et le temps de survie median pour les patients non guer…

Stage IV breast cancer: a population-based study about prognostic factors according to HER2 and HR status

International audience; We aim to describe trends in net survival (NS) and to assess the prognostic factors among women with de novo metastatic breast cancer (MBC) according to human epidermal growth factor receptor 2 (HER2) and hormone receptor (HR) status. Data on women suffering from de novo MBC and diagnosed from 1998 to 2009 were provided by the Côte-d'Or breast cancer registry. NS was described using the Pohar Perme estimator and prognostic factors were investigated in a generalised linear model. We identified 232 patients (mean age = 64.7). Median NS was 29.2 months, 1- and 5-year NS were 76% and 26% respectively. The survival trend in patients with HER2-positive tumours who did not …

Caractérisation de la thrombopénie immunologique associée aux hémopathies lymphoïdes à partir du registre des hémopathies malignes de Côte d’Or

Introduction Le purpura thrombopenique immunologique (PTI) est une cytopenie auto-immune (CAI) caracterisee par une diminution de la duree de vie des plaquettes liee a la fois a leur destruction peripherique par les macrophages spleniques et a un defaut de production medullaire. Son incidence est estimee entre 3 et 4/100 000 personnes-annees (PA). S’il est le plus souvent primaire, il est associe dans 15–20 % des cas a d’autres maladies auto-immunes ou a des hemopathies malignes. Pour ces dernieres, les associations les plus frequentes sont rapportees avec la leucemie lymphoide chronique (LLC), la leucemie a grands lymphocytes T granuleux (LGL) et le lymphome de Hodgkin (LH) mais les donnee…

Geographical Disparities in Multiple Myeloma Survival in Three French Regions

Abstract Introduction: Within the past decade, new therapeutic agents for Multiple Myeloma (MM) have improved survival of patients included in clinical trials. The aim of this study was to measure and compare characteristics and survival of patients with MM in different French regions in the general population. Methods: All incident cases with MM (ICD-O-3 codes, 9732/3 and) between January 1, 2008 and December 31, 2009 in the French population-based Hematological Malignancies registries (Calvados, Côte d'Or and Gironde) were included. The end point of follow-up was January 1, 2015. Specific clinical information was recorded from clinical charts. Incidence rates were calculated using estimat…

Leucocytosis and thrombosis at diagnosis are associated with poor survival in polycythaemia vera: a population-based study of 327 patients

Three hundred and twenty-seven patients from two population-based cohorts with an established diagnosis of polycythaemia vera were studied for prognostic risk factors for survival and leukaemia in a long-term survey. The relative survival (RS) was 72% and 46% at 10 and 20 years respectively, from the time of diagnosis. Multivariate analysis identified age >70 years, white blood cell count >13 × 10(9) /l and thrombo-embolism at diagnosis as independent risk factors. Patients with two or three of these factors had a 10 year RS of 26%, compared with 59% and 84% in patients with one and no risk factors, respectively. Age and leucocyte count are the main predicting factors for survival in polycy…

Time-to-cure and cure proportion in solid cancers in France. A population based study.

In cancer care, the cure proportion (P) and time-to-cure (TTC) are important indicators for practitioners, patients, and healthcare policy makers. The recent definition of TTC as the time at which the probability of belonging to the cured group reaches 95% was used for the first time.The data stem from the common database of French cancer registries including 335,358 solid tumours diagnosed between 1995 and 2009 at 27 sites. P and TTC were estimated through a flexible parametric net survival cure model for each cancer site, sex, and age at diagnosis with acceptable assumption of cure (excess mortality rate ≤0.05).TTC was ≤5 years and P was80% for skin melanoma and thyroid and testis cancers…

Leukocytosis is associated with poor survival but not with increased risk of thrombosis in essential thrombocythemia: a population-based study of 311 patients

Leukocytosis is associated with poor survival but not with increased risk of thrombosis in essential thrombocythemia: a population-based study of 311 patients

Worldwide trends in population-based survival for children, adolescents, and young adults diagnosed with leukaemia, by subtype, during 2000–14 (CONCORD-3): analysis of individual data from 258 cancer registries in 61 countries

BACKGROUND Leukaemias comprise a heterogenous group of haematological malignancies. In CONCORD-3, we analysed data for children (aged 0-14 years) and adults (aged 15-99 years) diagnosed with a haematological malignancy during 2000-14 in 61 countries. Here, we aimed to examine worldwide trends in survival from leukaemia, by age and morphology, in young patients (aged 0-24 years). METHODS We analysed data from 258 population-based cancer registries in 61 countries participating in CONCORD-3 that submitted data on patients diagnosed with leukaemia. We grouped patients by age as children (0-14 years), adolescents (15-19 years), and young adults (20-24 years). We categorised leukaemia subtypes a…

Risk assessment of second primary cancer according to histological subtype of non-Hodgkin lymphoma.

Non-Hodgkin lymphoma (NHL) represents a heterogeneous group of diseases that are known to carry a considerable risk of second primary cancer (SPC). However, little attention has been paid to SPC risk assessment according to NHL subtypes. Data from 10 French population-based cancer registries were used to establish a cohort of 7546 patients with a first diagnosis of NHL (eight subtypes) between 1989 and 2004. Standardized incidence ratios (SIRs) of metachronous SPC were estimated. Among the 7546 patients diagnosed with a NHL, the overall SPC risk was 25% higher than that in the reference population (SIR = 1.25, 95% confidence interval 1.15–1.36). In univariate analysis, the SPC risk differed…

Trends in Incidence and Survival of Myeloid Malignancies Since 1980, in the Côte D'or Department, Burgundy, France.

Abstract Abstract 3122 Poster Board III-59 Objective The Registry of Haematological Malignancies (HM) has been established on January 1st, 1980 in the department of Côte d'Or (pop 500 000 inhabitants). It was the first specialized registry in haematology in the world. During the course of 25 years (1980-2004), 5026 cases of HM were recorded including 1553 Myeloid malignancies (MM) in which entities not initially considered as malignant were taken in account such as Myelodysplastic syndrome (MDS) and some Myeloproliferative neoplasms (MPN). This allow us to present trends in incidence and survival of myeloid malignancies by entities since 1980. Method MM diagnosed in the population between …

Leukocytosis and Thrombosis At Diagnosis Are Associated with Poor Survival in Polycythemia Vera: A Population-Based Study on 327 Patients

Abstract Abstract 2819 Introduction Polycythemia Vera (PV) is a myeloproliferative neoplasm with a long life expectancy. Consequently, long-term follow-up studies that focus on survival of PV patients must be conducted in order to register a sufficient number of events to provide informative results and statistical analyses. Most of the published studies are single-institution that focused on patients referred to hospitals, which does not necessarily reflect the course of PV in the general population. In these studies, the median age at diagnosis is younger than usually noted for PV patients in population-based studies. Moreover, survival is generally based on observed mortality, a good but…

Second Cancer Incidence Among Chronic Lymphocytic Leukemia (CLL) Patients: A French Population-Based Study

Abstract Introduction. The risk of developing a second primary cancer (SPC) is increased in patients with CLL. The mechanisms explaining this association could be related to lifestyle, environment, host factors or interactions or other influences. We conducted an epidemiological study based on 10 French registries and evaluated the risk of developing SPC in patients with CLL. Methods. Data from French population-based registries were used to establish a cohort of all patients diagnosed with a first cancer between 1989 and 2004 and followed-up until December 31, 2007. The person-year approach was used to estimate Standardized Incidence Ratios (SIRs) and Excess Absolute Risks (EARs) of metach…