Title :

Gastrointestinal Behcet''s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review.

Author(s) :

Wesner, Nadege [Auteur]
CHU Saint-Antoine [AP-HP]
Drevon, Louis [Auteur]
CHU Saint-Antoine [AP-HP]
Guedon, Alexis [Auteur]
Institut Pierre Louis d'Epidémiologie et de Santé Publique [iPLESP]
Fraison, Jean-Baptiste [Auteur]
Terrier, Benjamin [Auteur]
Hôpital Ambroise Paré [AP-HP]
Trad, Salim [Auteur]
Hôpital Ambroise Paré [AP-HP]
Kahn, Jean-Emmannuel [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Aouba, Achille [Auteur]
CHU Caen
Gillard, Jerome [Auteur]
Centre Hospitalier Jura Sud [Lons-le-Saunier] [CH Jura Sud]
Ponsoye, Matthieu [Auteur]
Hôpital Ambroise Paré [AP-HP]
Hanslik, Thomas [Auteur]
Hôpital Ambroise Paré [AP-HP]
Gourguechon, Clement [Auteur]
CHU Amiens-Picardie
Liozon, Eric [Auteur]
Service de Médecine interne A et polyclinique médicale [CHU Limoges]
Laribi, Kamel [Auteur]
Centre Hospitalier Le Mans (CH Le Mans)
Rossignol, Julien [Auteur]
Hôpital Necker - Enfants Malades [AP-HP]
Hermine, Olivier [Auteur]
Hôpital Necker - Enfants Malades [AP-HP]
Seksik, Philippe [Auteur]
CHU Saint-Antoine [AP-HP]
Ades, Lionel [Auteur]
Hopital Saint-Louis [AP-HP] [AP-HP]
Carrat, Fabrice [Auteur]
Institut Pierre Louis d'Epidémiologie et de Santé Publique [iPLESP]
Fenaux, Pierre [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Mekinian, Arsene [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Fain, Olivier [Auteur]
CHU Pitié-Salpêtrière [AP-HP]

Journal title :

Leukemia & lymphoma

Abbreviated title :

Leuk. Lymphoma

Volume number :

60

Pages :

1-7

Publication date :

2019

ISSN :

1029-2403

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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 Behcet'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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Inserm
Université de Lille
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Research team(s) :

Immunity, inflammation and fibrsis in auto and allo-reactivity

Submission date :

2019-03-01T14:17:32Z
2024-04-08T11:52:06Z