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Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review.
Wesner, Nadege; Drevon, Louis; Guedon, Alexis; Fraison, Jean Baptiste; Terrier, Benjamin; Trad, Salim; Kahn, Jean Emmanuel; Aouba, Achille; Gillard, Jerome; Ponsoye, Matthieu; Hanslik, Thomas; Gourguechon, Clement; Liozon, Eric; Laribi, Kamel; Rossignol, Julien; Hermine, Olivier; Seksik, Philippe; Adès, Lionel; Carrat, Fabrice; Fenaux, Pierre; Mekinian, Arsène; Fain, Olivier.
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  • Wesner N; a Department of Internal Medicine Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris , Hôpital Saint-Antoine , Paris , France.
  • Drevon L; b INSERM U938, Centre de Recherche Saint-Antoine (CRSA) , Sorbonne Universités , Paris , France.
  • Guedon A; a Department of Internal Medicine Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris , Hôpital Saint-Antoine , Paris , France.
  • Fraison JB; b INSERM U938, Centre de Recherche Saint-Antoine (CRSA) , Sorbonne Universités , Paris , France.
  • Terrier B; c INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique IPLESP, AP-HP, Hôpital Saint Antoine , Sorbonne Université , Paris , France.
  • Trad S; d Department of Internal Medicine , Hôpital Sète , France.
  • Kahn JE; e Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris , Hôpital Ambroise Paré , Boulogne Billancourt , France.
  • Aouba A; e Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris , Hôpital Ambroise Paré , Boulogne Billancourt , France.
  • Gillard J; f Department of Internal Medicine , Hôpital Foch , Suresnes , France.
  • Ponsoye M; g Department of Internal Medicine, CH Caen , Caen , France.
  • Hanslik T; h Department of Rheumatology, CH Lons le Saunie , Lons le Saunier , France.
  • Gourguechon C; e Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris , Hôpital Ambroise Paré , Boulogne Billancourt , France.
  • Liozon E; e Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris , Hôpital Ambroise Paré , Boulogne Billancourt , France.
  • Laribi K; i Department of Internal Medicine, CHU Amiens-Picardie , Amiens , France.
  • Rossignol J; j Department of Internal Medicine, CHU Limoges , Limoges , France.
  • Hermine O; k Department of Hematology , Centre Hospitalier Le Mans , Le Mans , France.
  • Seksik P; l Department of Hematology, Assistance Publique-Hôpitaux de Paris , Hôpital Necker Enfants Malades , Paris , France.
  • Adès L; l Department of Hematology, Assistance Publique-Hôpitaux de Paris , Hôpital Necker Enfants Malades , Paris , France.
  • Carrat F; m Department of Gastro Enterology Hopital Saint Antoine , Sorbonne Université, APHP, Hôpital Saint-Antoine , Paris , France.
  • Fenaux P; n Department of Hematology, Assistance Publique-Hôpitaux de Paris , Hôpital Saint Louis , Paris , France.
  • Mekinian A; c INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique IPLESP, AP-HP, Hôpital Saint Antoine , Sorbonne Université , Paris , France.
  • Fain O; b INSERM U938, Centre de Recherche Saint-Antoine (CRSA) , Sorbonne Universités , Paris , France.
Leuk Lymphoma ; 60(7): 1782-1788, 2019 07.
Article en En | MEDLINE | ID: mdl-30457024
We report the 11 cases of +8-MDS/MPN associated with Behcet's-like syndrome and compare them with Behcet's disease and Crohn's disease, pool with literature cases for analysis. Data for patients with +8-MDS/MPN and Behçet's-like syndrome were collected from MINHEMON. Eleven patients had Behcet's-like syndrome and +8-MDS/MPN (median age 75 years [IQR 65-87]; M/F ratio 0.8). MDS and Behcet's-like syndrome were diagnosed at the same time (7/11, 64%). By comparison with 63 patients with idiopathic Behcet's disease without associated MDS, those with Behcet's-like syndrome and +8-MDS/MPN were older (median 75 vs 48 years; p = .0003) and had less pseudofolliculitis (11% vs 62%; p = .0045) and ocular impairment (0% vs 52%; p = .0008), but more frequent gastrointestinal involvement (60% vs 13%; p = .0005). By comparison with Crohn's disease, 39 patients with Behcet's-like syndrome and +8-MDS/MPN were significantly older (median 72 [53-78] vs 36 [27-45] years; p = .0002) and more frequently had oral aphtosis (97% vs 5%, p < .0001), skin features (50% vs 10%, p = .0005) and arthralgia (63% vs 20%, p = .03). Median survival did not differ between patients with Behcet's-like syndrome and +8-MDS/MPN and those with +8-MDS/MPN (n = 103) (47 vs 34 months, p = .61). AML-free survival did not differ between patients with MDS/MPN with and without Behcet's-like syndrome (p = .29). MDS/MPN with trisomy 8 can be associated with particular phenotype of ulcerative digestive disease resembling Behcet's or Crohn's disease and should be considered a single disease.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trisomía / Síndromes Mielodisplásicos / Síndrome de Behçet / Enfermedades Mielodisplásicas-Mieloproliferativas / Enfermedades Gastrointestinales Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Leuk Lymphoma Asunto de la revista: HEMATOLOGIA / NEOPLASIAS Año: 2019 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trisomía / Síndromes Mielodisplásicos / Síndrome de Behçet / Enfermedades Mielodisplásicas-Mieloproliferativas / Enfermedades Gastrointestinales Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Leuk Lymphoma Asunto de la revista: HEMATOLOGIA / NEOPLASIAS Año: 2019 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Estados Unidos