• Leukemia & lymphoma · Jul 2019

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

    • Nadege Wesner, Louis Drevon, Alexis Guedon, Jean Baptiste Fraison, Benjamin Terrier, Salim Trad, Jean Emmanuel Kahn, Achille Aouba, Jerome Gillard, Matthieu Ponsoye, Thomas Hanslik, Clement Gourguechon, Eric Liozon, Kamel Laribi, Julien Rossignol, Olivier Hermine, Philippe Seksik, Lionel Adès, Fabrice Carrat, Pierre Fenaux, Arsène Mekinian, Olivier Fain, and On behalf GFM and MINHEMON (French Network of dysimmune disorders associated with hemopathies).
    • a Department of Internal Medicine Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris , Hôpital Saint-Antoine , Paris , France.
    • Leuk. Lymphoma. 2019 Jul 1; 60 (7): 1782-1788.

    AbstractWe 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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