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

AbstractWe report the 11 cases of +8-MDS/MPN associated with Behcet’s-like syndrome and compare them with Behcet’s diseaseand 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 ratio0.8). MDS and Behcet’s-like syndrome were diagnosed at the same time (7/11, 64%). By comparison with 63 patients with idiopathic Behcet’s diseasewithout 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 aphtosi...