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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]

Objective: We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated with inflammatory and autoimmune diseases (IADs). Method: Data for 21 patients with trisomy 8‐MDS/MPN and IADs were analyzed and compared to 103 patients with trisomy 8‐MDS/MPN without IADs. Results: The median age of MDS/MPN patients with IADs was 67 [59‐80]. The IADs were Behçet's‐like disease in 11 (52%) patients, inflammatory arthritis in 4 (19%) and Sjögren's syndrome, autoimmune hemolytic anemia, aseptic abscess, periarteritis nodosa, Sweet's syndrome and unclassified vasculitis in one patient each. Overall, 17/21 (81%) patients with IADs received treatment (88% with steroids), with complete and partial response in 7/17 (35%) and 8/17 (47%), respectively. The effect of MDS treatment on IADs could be assessed in seven patients receiving azacytidine: five achieved remission and two partial response. As compared with the 103 trisomy 8‐MDS/MPN cases without IADs, those with IADs were more often non‐European (P = 0.005) and had poor karyotype (P < 0.001). We found no difference in overall survival or acute myeloid leukemia progression between trisomy 8‐associated MDS/MPN with and without IADs. Conclusion: The spectrum of IADs associated with trisomy 8‐positive MDS/MPN is dominated by Behçet's‐like disease. Steroid therapy is effective, but mostly sparing therapies are necessary. Azacytidine could be an effective alternative. [ABSTRACT FROM AUTHOR]

Background: Immune checkpoint inhibitors (ICIs) may induce overlapping myositis/myasthenia gravis (MG) features, sparking current debate about pathophysiology and management of this emerging disease entity. We aimed to clarify whether ICI-induced (ir-) myositis and ir-MG represent distinct diseases or exist concurrently., Methods: We performed a retrospective multicenter cohort study. Using the Paris University Hospitals database (n = 2,910,417), we screened all patients with International Classification of Diseases codes or free text related to myositis/MG signs and ICI (n = 620). 'Ir-MG signs' were defined by fatigability, repetitive nerve stimulation (RNS) decrement, and/or acetylcholine receptor antibodies (AChR Abs)., Findings: Ir-MG signs were never observed in the absence of ir-myositis (pathological diagnosis (n = 12/14) or CK levels >8000 U/L (n = 2/14)). Among ir-myositis patients, fatigability (2%; n = 1/62) and RNS decrement (2%; n = 1/41) were demonstrated only in one patient with pre-existing MG. AChR Abs testing yielded positive results in 26% of ir-myositis patients (n = 14/53). We revealed that test results were already positive prior to ICI therapy (n = 8/9). Clinically, ir-myositis frequently presented with "MG-like" oculomotor disease (50%; n = 31/62), bulbar dysfunction affecting speech (29%; n = 18/62) and swallowing (42%; n = 26/62), and respiratory disorders (53%; n = 33/62). Extraocular and diaphragm muscles necropsies disclosed intense muscle inflammation (100%; n = 5/5)., Interpretation: In our extensive database, we found no evidence of isolated ir-MG, nor of clear neuromuscular junction dysfunction in ir-myositis. These findings suggest that patients with ir-MG suspicion frequently have ir-myositis and ir-MG might be rare. "MG-like" symptoms may stem from ir-myositis-specific predilection for oculo-bulbo-respiratory musculature. Indeed, we revealed florid inflammatory infiltration of the oculomotor and respiratory muscles. Additional studies are needed to confirm these results and to elucidate the role of pre-existing AChR Abs in ir-myositis., Funding: None., Competing Interests: None of the authors received financial support for the submitted work. CA reports one patent planned in the field of management of immune checkpoint inhibitors toxicities. BA reports a research grant from MSD Avenir, consulting fees from Novartis, Astellas, and Sanofi, personal honorarium from Sanofi, AstraZeneca, BMS, MSD and Astellas, and support for attending meetings and/or travel from Janssen, MSD, Pfizer, IPSEN Pharma and Takeda. MT reports a grant from Sanofi, consulting fees from Servier, Novocure and NH TherAguiX, personal honorarium from Servier, Novocure and ONO, and support for attending meetings and/or travel from Servier. MT participated on a Data Safety Monitoring or Advisory Board for Servier. MCB reports personal honorarium, and support for attending meetings and/or travel from Novartis. SE reports consulting fees from Bayer, Amgen and Ipsen, and personal honorarium from AstraZeneca, BMS, Philips, General Electric, and Eisai. FT reports non-personal consulting fees from MSD, Novartis, and GSK. JES reports personal consulting fees from AstraZeneca, BeiGene, BMS and Novartis, several patents planned, issued or pending in the field of management of immune checkpoint inhibitors toxicities. YA received research funding from Sanofi and Association Recherche contre le Cancer, consulting fees from BMS, personal honorarium from RE-IMAGINE Health Agency and CSL Behring SA, and support for attending meetings and/or travel from CSL Behring SA and Boehringer Ingelheim France. YA had several patents planned, issued or pending in the field of management of immune checkpoint inhibitors toxicities., (© 2025 The Authors.)