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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. [ABSTRACT FROM AUTHOR]

Abstract: Background: Biotherapies targeting TNFα were proven to be effective in the most severe cases of Crohn''s Disease, a chronic granulomatous inflammatory bowel disease that can involve any portion of the digestive tract. The tolerance of anti-TNFα therapy is usually good, although several infectious complications have been reported with these drugs. Methods: We report a case of a Crohn''s disease patient who developed pulmonary cryptococcosis following chicken manure exposition while he received adalimumab and azathioprine. Case: A 54-year-old man, with history of severe Crohn''s disease and ankylosing spondylitis, was admitted for diarrhea and abdominal pain under azathioprine treatment. In December 2010, he was treated with oral prednisone (1mg/kg/day), but Crohn''s disease relapsed when prednisone dose was lower than 30mg/a day. The patient was then treated with adalimumab, but six weeks later he developed severe pulmonary cryptococcosis. The patient experienced a good outcome under antifungal therapy. We retrospectively found a high exposure to chicken manure in the last weeks. Conclusion: Cryptococcosis is an opportunistic infection that can occur under anti-TNFα therapy. The environmental exposure to Cryptococcus spp. (in particular in chicken manure) is a source of contamination. Avoiding exposition to bird manure should be a recommendation for patients who are living in rural areas. [Copyright &y& Elsevier]