Structuration nationale pour la prise en charge des lymphomes à petites (sprue réfractaire de type II) et grandes cellules associés à la maladie cœliaque

université Paris-Descartes Inserm U793, faculté Necker 156, rue de Vaugirard F-75015 Paris France
Oncologie (Impact Factor: 0.08). 05/2008; 10(6):421-424. DOI: 10.1007/s10269-008-0890-7

ABSTRACT RésuméLes lymphomes de bas et de haut grades sont des complications rares et graves de la maladie cœliaque. Les outils et critères
diagnostiques de ces lymphomes intestinaux sont récents, très spécialisés et insuffisamment diffusés. Il n’existe pas de traitement

ButsHomogénéiser et diffuser les outils et critères diagnostiques, le suivi et la prise en charge thérapeutique.

Patients et méthodesCréation d’un réseau national constitué de 16 centres nationaux associés. L’expertise et les plateformes techniques de l’équipe
coordinatrice (études phénotypiques, histologiques, cytogénétiques et moléculaires) seront à la disposition de toutes les
équipes du réseau. Les données cliniques et biologiques des dossiers médicaux des patients seront centralisées et informatisées
dans une base de données et discutées au cours des réunions de concertation pluridisciplinaires nationales.

ConclusionLymphocœliaque devrait permettre une meilleure prise en charge diagnostique et thérapeutique des patients atteints de lymphomes
associés à la maladie cœliaque.

AbstractLow-grade and overt lymphomas are rare and severe complications of the celiac disease. The diagnostic techniques and criteria
to be used for these intestinal lymphomas are very specific; they have been developed recently and remain insufficiently known.
Efficient treatment does not exist.

AimsTo homogenize and make available for all the diagnostic techniques and criteria of these complications, their follow-up and
therapeutic management.

Patients and methodsOrganization of a national network that will include 16 national centres. The technical means, skills and experience of the
coordinating group (for phenotyping, histological, cytogenetic and molecular studies) will be available to all centres of
the network. Clinical and biological data of the patients will be centralized and recorded on computer medical files and discussed
during multidisciplinary committee meetings.

ConclusionThe “Lymphocœliaque” organization should improve the diagnosis and therapeutic management of celiac patients with lymphomas.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Studies of cancer risk in celiac disease (CD) or dermatitis herpetiformis (DH) indicate increased risks for malignant lymphoma and occasionally other neoplasms, but are characterized by small numbers, lack of systematic cancer assessment, and subjects identified from referral institutions. By using Swedish population-based inpatient and cancer registry data, we followed-up 12,000 subjects with CD or DH, and evaluated cancer incidence by using standardized incidence ratios (SIR). Adults (but not children and adolescents) with CD had an elevated overall risk for cancer (SIR = 1.3) that declined with time and eventually reached unity. Elevated risks were found for malignant lymphomas, small-intestinal, oropharyngeal, esophageal, large intestinal, hepatobiliary, and pancreatic carcinomas. The excess occurrence of malignant lymphomas was confined to adults, decreased with time of follow-up evaluation, and decreased over successive calendar periods. Decreased risks were found for breast cancer. Subjects with DH had a slightly increased overall cancer risk (SIR = 1.2) owing to excesses of malignant lymphoma and leukemia, but no increases of gastrointestinal carcinomas. Albeit increased, the relative risks for lymphomas and gastrointestinal cancers in this study are lower (and declining) than in most previous reports. The overall cancer risk is only moderately increased, and nonelevated during childhood and adolescence.
    Gastroenterology 11/2002; 123(5):1428-35. DOI:10.1053/gast.2002.36585 · 13.93 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: No data on mortality in celiac disease are currently available in southern Europe. Our aim was to evaluate mortality and the cause of death in adult celiac disease in a Mediterranean area. In all, 228 adults with celiac disease were histologically diagnosed in our department from 1980 to 1997. Full information on their state of health was obtained in 216 of 228 patients. A tabulation of patient-years at risk was constructed in terms of age at diagnosis and the interval from diagnosis. Standardized mortality ratio was calculated by dividing the number of observed deaths by the number of expected deaths. Twelve deaths were observed, whereas 3.12 deaths were expected (SMR = 3.8; 95% CI 2-7). The increased mortality was mainly observed within four years from diagnosis (8 observed; 1.4 expected) (SMR = 5.8; 95% CI 2.5-11.5). Twelve tumors were observed (six lymphomas). In conclusion, mortality from adult celiac disease in our geographical area is increased compared with the general population, and this increased risk seems due to non-Hodgkin's lymphoma.
    Digestive Diseases and Sciences 01/2000; 44(12):2538-41. DOI:10.1023/A:1026655609906 · 2.55 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Refractory coeliac sprue (RCS) with an immunophenotypically aberrant clonal intraepithelial lymphocyte (IEL) population is considered a cryptic form of intestinal T cell lymphoma. To investigate the distribution of the abnormal and monoclonal IEL population in the digestive tract of RCS patients. We compared the frequency of lymphocytic gastritis (LG) and lymphocytic colitis (LC), together with IEL phenotype and T cell clonality, in gastric and colonic samples from 15 adults with RCS (all with aberrant CD3 intracytoplasmic(+) surface(-) CD8(-) clonal IELs on duodenojejunal biopsies), 18 patients with active coeliac disease (ACD), and 10 patients with coeliac disease (CD) on a gluten free diet (GFD-CD) by means of immunohistochemistry and multiplex polymerase chain reaction amplification of the T cell receptor gamma gene (TCR-gamma) rearrangement. Blood samples of nine RCS patients were also tested for clonality. LG was found in 9/14 (64%), 11/18 (61%), and 3/10 (30%) patients with RCS, ACD, and GFD-CD, respectively, while LC was found in 6/11 (55%), 3/4 (75%), and 2/3 (66%) patients. Contrary to CD, all samples from patients with LG and LC showed an aberrant IEL phenotype. Monoclonal TCR-gamma rearrangements were detected in 8/13 (62%), 8/10 (80%), and 4/9 (44%) of gastric, colonic, and blood samples, respectively, from RCS patients, while in CD patients such rearrangements were only found in 2/25 (8%) gastric samples. The immunophenotypically aberrant monoclonal IEL population present in the small intestine of patients with RCS frequently disseminates to the blood and the entire gastrointestinal epithelium, suggesting that this is a diffuse gastrointestinal disease.
    Gut 03/2003; 52(2):205-11. DOI:10.1136/gut.52.2.205 · 13.32 Impact Factor
Show more