Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8
Myelodysplastic syndromes (MDSs) are a group of myeloid neoplasms characterized by blood cell deformation and dysfunction, and MDS with trisomy 8 is closely linked with intestinal Behçet’s-like diseases. Intestinal Behçet’s-like disease is refractory to conventional therapies, including prednisolone...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Taylor & Francis Group
2019-11-01
|
| Series: | Immunological Medicine |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/25785826.2019.1687251 |
| _version_ | 1818064529606574080 |
|---|---|
| author | Nahoko Tanaka Hirotake Sakuraba Hiroto Hiraga Ko Mayama Hidezumi Kikuchi Dai Kishida Yui Akemoto Keisuke Hasui Shinji Ota Rina Watanabe Yasuhisa Murai Takato Maeda Kosuke Kamata Tetsuya Tatsuta Manabu Sawaya Daisuke Chinda Tatsuya Mikami Kazufumi Yamagata Yoh Ishiguro Masanori Tanaka Shinsaku Fukuda |
| author_facet | Nahoko Tanaka Hirotake Sakuraba Hiroto Hiraga Ko Mayama Hidezumi Kikuchi Dai Kishida Yui Akemoto Keisuke Hasui Shinji Ota Rina Watanabe Yasuhisa Murai Takato Maeda Kosuke Kamata Tetsuya Tatsuta Manabu Sawaya Daisuke Chinda Tatsuya Mikami Kazufumi Yamagata Yoh Ishiguro Masanori Tanaka Shinsaku Fukuda |
| author_sort | Nahoko Tanaka |
| collection | DOAJ |
| description | Myelodysplastic syndromes (MDSs) are a group of myeloid neoplasms characterized by blood cell deformation and dysfunction, and MDS with trisomy 8 is closely linked with intestinal Behçet’s-like diseases. Intestinal Behçet’s-like disease is refractory to conventional therapies, including prednisolone, immunomodulators, and anti-tumor necrosis factor α agents. Here, we describe a 56-year-old woman with intestinal Behçet’s-like disease ascribed to MDS with trisomy 8 who had multiple intractable intestinal ulcers. She presented with periodic fever and abdominal pain. The genetic analysis showed a heterozygous E148Q mutation in the Mediterranean fever gene. The patient did not tolerate treatment with colchicine because of diarrhea; therefore, azacitidine therapy was initiated. One cycle of azacitidine therapy improved the multiple intestinal ulcers, and the periodic fever and abdominal pain gradually disappeared. After eight cycles of azacitidine therapy, ileocolonoscopy, histological assessment and capsule endoscopy revealed mucosal healing. Azacitidine therapy was continued, and mucosal healing was maintained for more than 2 years. This case suggests that azacitidine therapy which has immunoregulatory effects and epigenetic modulations, might control intestinal Behçet’s-like disease associated with MDS involving trisomy 8. |
| first_indexed | 2024-12-10T14:37:27Z |
| format | Article |
| id | doaj.art-e96bd3e1e97e4b3985e8b1b1c18a6668 |
| institution | Directory Open Access Journal |
| issn | 2578-5826 |
| language | English |
| last_indexed | 2024-12-10T14:37:27Z |
| publishDate | 2019-11-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Immunological Medicine |
| spelling | doaj.art-e96bd3e1e97e4b3985e8b1b1c18a66682022-12-22T01:44:47ZengTaylor & Francis GroupImmunological Medicine2578-58262019-11-01001710.1080/25785826.2019.16872511687251Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8Nahoko Tanaka0Hirotake Sakuraba1Hiroto Hiraga2Ko Mayama3Hidezumi Kikuchi4Dai Kishida5Yui Akemoto6Keisuke Hasui7Shinji Ota8Rina Watanabe9Yasuhisa Murai10Takato Maeda11Kosuke Kamata12Tetsuya Tatsuta13Manabu Sawaya14Daisuke Chinda15Tatsuya Mikami16Kazufumi Yamagata17Yoh Ishiguro18Masanori Tanaka19Shinsaku Fukuda20Hirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineShinshu University School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki University Graduate School of MedicineHirosaki National Hospital, National Hospital OrganizationHirosaki Municipal HospitalHirosaki University Graduate School of MedicineMyelodysplastic syndromes (MDSs) are a group of myeloid neoplasms characterized by blood cell deformation and dysfunction, and MDS with trisomy 8 is closely linked with intestinal Behçet’s-like diseases. Intestinal Behçet’s-like disease is refractory to conventional therapies, including prednisolone, immunomodulators, and anti-tumor necrosis factor α agents. Here, we describe a 56-year-old woman with intestinal Behçet’s-like disease ascribed to MDS with trisomy 8 who had multiple intractable intestinal ulcers. She presented with periodic fever and abdominal pain. The genetic analysis showed a heterozygous E148Q mutation in the Mediterranean fever gene. The patient did not tolerate treatment with colchicine because of diarrhea; therefore, azacitidine therapy was initiated. One cycle of azacitidine therapy improved the multiple intestinal ulcers, and the periodic fever and abdominal pain gradually disappeared. After eight cycles of azacitidine therapy, ileocolonoscopy, histological assessment and capsule endoscopy revealed mucosal healing. Azacitidine therapy was continued, and mucosal healing was maintained for more than 2 years. This case suggests that azacitidine therapy which has immunoregulatory effects and epigenetic modulations, might control intestinal Behçet’s-like disease associated with MDS involving trisomy 8.http://dx.doi.org/10.1080/25785826.2019.1687251myelodysplastic syndrometrisomy 8behçet’s diseaseazacitidinemucosal healing |
| spellingShingle | Nahoko Tanaka Hirotake Sakuraba Hiroto Hiraga Ko Mayama Hidezumi Kikuchi Dai Kishida Yui Akemoto Keisuke Hasui Shinji Ota Rina Watanabe Yasuhisa Murai Takato Maeda Kosuke Kamata Tetsuya Tatsuta Manabu Sawaya Daisuke Chinda Tatsuya Mikami Kazufumi Yamagata Yoh Ishiguro Masanori Tanaka Shinsaku Fukuda Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 Immunological Medicine myelodysplastic syndrome trisomy 8 behçet’s disease azacitidine mucosal healing |
| title | Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| title_full | Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| title_fullStr | Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| title_full_unstemmed | Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| title_short | Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| title_sort | long term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal behcet s like disease accompanied with myelodysplastic syndrome involving trisomy 8 |
| topic | myelodysplastic syndrome trisomy 8 behçet’s disease azacitidine mucosal healing |
| url | http://dx.doi.org/10.1080/25785826.2019.1687251 |
| work_keys_str_mv | AT nahokotanaka longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT hirotakesakuraba longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT hirotohiraga longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT komayama longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT hidezumikikuchi longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT daikishida longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT yuiakemoto longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT keisukehasui longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT shinjiota longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT rinawatanabe longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT yasuhisamurai longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT takatomaeda longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT kosukekamata longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT tetsuyatatsuta longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT manabusawaya longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT daisukechinda longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT tatsuyamikami longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT kazufumiyamagata longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT yohishiguro longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT masanoritanaka longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 AT shinsakufukuda longtermmaintenanceofthemucosalhealinginducedbyazacitidinetherapyinapatientwithintestinalbehcetslikediseaseaccompaniedwithmyelodysplasticsyndromeinvolvingtrisomy8 |