Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8 : a French case series and literature review
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:60 |
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| Enthalten in: |
Leukemia & lymphoma - 60(2019), 7 vom: 25. Juli, Seite 1782-1788 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Wesner, Nadege [VerfasserIn] |
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| Links: |
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| Themen: |
Behcet’s disease |
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| Anmerkungen: |
Date Completed 28.07.2020 Date Revised 28.07.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1080/10428194.2018.1542152 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM290887143 |
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| 245 | 1 | 0 | |a Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8 |b a French case series and literature review |
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| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Behcet’s disease | |
| 650 | 4 | |a outcome | |
| 650 | 4 | |a treatment | |
| 650 | 4 | |a trisomy 8-positive myelodysplastic syndrome | |
| 700 | 1 | |a Drevon, Louis |e verfasserin |4 aut | |
| 700 | 1 | |a Guedon, Alexis |e verfasserin |4 aut | |
| 700 | 1 | |a Fraison, Jean Baptiste |e verfasserin |4 aut | |
| 700 | 1 | |a Terrier, Benjamin |e verfasserin |4 aut | |
| 700 | 1 | |a Trad, Salim |e verfasserin |4 aut | |
| 700 | 1 | |a Kahn, Jean Emmanuel |e verfasserin |4 aut | |
| 700 | 1 | |a Aouba, Achille |e verfasserin |4 aut | |
| 700 | 1 | |a Gillard, Jerome |e verfasserin |4 aut | |
| 700 | 1 | |a Ponsoye, Matthieu |e verfasserin |4 aut | |
| 700 | 1 | |a Hanslik, Thomas |e verfasserin |4 aut | |
| 700 | 1 | |a Gourguechon, Clement |e verfasserin |4 aut | |
| 700 | 1 | |a Liozon, Eric |e verfasserin |4 aut | |
| 700 | 1 | |a Laribi, Kamel |e verfasserin |4 aut | |
| 700 | 1 | |a Rossignol, Julien |e verfasserin |4 aut | |
| 700 | 1 | |a Hermine, Olivier |e verfasserin |4 aut | |
| 700 | 1 | |a Seksik, Philippe |e verfasserin |4 aut | |
| 700 | 1 | |a Adès, Lionel |e verfasserin |4 aut | |
| 700 | 1 | |a Carrat, Fabrice |e verfasserin |4 aut | |
| 700 | 1 | |a Fenaux, Pierre |e verfasserin |4 aut | |
| 700 | 1 | |a Mekinian, Arsène |e verfasserin |4 aut | |
| 700 | 1 | |a Fain, Olivier |e verfasserin |4 aut | |
| 700 | 0 | |a On behalf GFM and MINHEMON (French Network of dysimmune disorders associated with hemopathies) |e verfasserin |4 aut | |
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