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

Annals of Hematology
Umut YilmazAykut Ferhat Celik

Abstract

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 glucoc...Continue Reading

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Citations

Jun 15, 2020·Annals of Hematology·Ivan KrečakVelka Gverić-Krečak
May 22, 2021·Pediatric Blood & Cancer·Asaf D YanirShlomit Barzilai Birenboim
Jul 30, 2021·Journal of Inflammation Research·Yesim OzgulerGulen Hatemi
Oct 13, 2021·BMC Gastroenterology·Xiaofen ZhangChaohui Yu

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