How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review

Yılmaz U., Ar M. C., Bavunoğlu I., Erzin Y. Z., Hatemi A. İ., Yıldırım S., ...More

Annals Of Hematology, vol.99, no.6, pp.1193-1203, 2020 (SCI-Expanded)

  • Publication Type: Article / Article
  • Volume: 99 Issue: 6
  • Publication Date: 2020
  • Doi Number: 10.1007/s00277-020-03951-5.
  • Journal Name: Annals Of Hematology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, CINAHL, EMBASE, MEDLINE
  • Page Numbers: pp.1193-1203
  • Istanbul University Affiliated: No


The association between myelodysplastic syndrome (MDS) and Behçet syndrome (BS) is recognized for over 25 years. High frequency of trisomy 8 and intestinal ulcers are striking features of this association. There are no recommendations for how these patients should be treated. A systematic literature review was performed in PubMed using the keyword combination “(((((intestinal) OR gastrointestinal) OR ulcer) OR Behcet*)) AND ((myelodysplastic syndrome) OR MDS)” in March 2019. Our aim was to gain insight regarding clinical responses to individual treatment modalities. A recent case was also presented and included in the analysis. Data from 41 articles reporting on a total of 53 patients carried adequate information to assess treatment responses. Glucocorticoids provided benefit in 23 of 43 patients. Azacitidine, decitabine, thalidomide, and cyclosporine contributed to a clinical improvement in 4/6, 2/3, 3/4, and 5/8 patients respectively. Hematopoietic stem cell transplantation was successful in 9 of 13 patients. With the use of TNF inhibitors, azathioprine, and mesalamine derivatives, clinical improvement was observed in 3/11, 0/4, and 6/18 patients respectively. Patients with MDS and BS-like features who are resistant to glucocorticoids have so far benefited more from treatment approaches directed at MDS, rather than the immunosuppressive agents used for BS.