A Case of Myelodysplastic Syndrome with Intestinal Behçet’s Disease-Like Symptoms Treated by Prednisolone and Azacitidine

Abstract

Patient: Female, 68Final Diagnosis: Myelodysplastic Syndrome with Intestinal Behçet’s Disease-Like SymptomsSymptoms: Abdominal pain • fever • oral ulcerMedication: —Clinical Procedure: CT • bone marrow examination • colonoscopySpecialty: HematologyObjective:Rare diseaseBackground:Intestinal Behçet’s disease-like symptoms are rare complications of myelodysplastic syndrome and are often refractory to immunosuppressive therapies. We described a case of myelodysplastic syndrome complicated by Behçet’s disease-like symptoms treated with prednisolone and azacitidine.Case Report:A 68-year-old Japanese woman was admitted to our hospital because of persistent high fever and lower abdominal pain. Oral ulcerations developed after admission, and multiple ulcers were found in her terminal ileum by endoscopic examination. She was diagnosed with myelodysplastic syndrome with trisomy 8 by bone marrow examination. Her symptoms diminished after administration of prednisolone, but relapsed afterwards. She began azacitidine therapy and her symptoms have been controlled for at least 10 months.Conclusions:This case might suggest the possibility of azacitidine as a treatment option for myelodysplastic syndrome complicated by Behçet’s disease-like symptoms.

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

“…In our case, AZA treatment induced clinical and endoscopic remission. A few reports showed the efficacy of AZA for treating intestinal Behçet's-like disease associated with MDS involving trisomy 8 (Table 2) [13][14][15][16]. However, there was no report that AZA treatment induced and maintained complete both clinical and endoscopic remission of intestinal lesions ascribed to MDS involving trisomy 8.…”

The burden of autoimmunity in myelodysplastic syndromes

“…As regards azacytidine, 10 years ago, the first reports described MDS patients showing a complete response of deep neutrophilic dermatosis and an improvement of SLE after treatment. Also BD was shown to significantly improve with azacytidine . In 2 following reports describing 3 patients each, different autoimmune conditions were reported to respond to this hypomethylating agent, such as Sweet's syndrome, polyarthritis, and vasculitis as well as rheumatoid arthritis and fluctuating lupus‐like symptoms .…”

“…111,112 In 2 following reports describing 3 patients each, different autoimmune conditions were reported to respond to this hypomethylating agent, such as Sweet's syndrome, polyarthritis, and vasculitis 117,118 as well as rheumatoid arthritis and fluctuating lupus-like symptoms. 112 In most of these patients, the autoimmune conditions were resistant to several lines of immunosuppressive therapy, and their improve- cycles. Furthermore, although less than 50% of the patients received more than 6 cycles, only 3 relapses were observed.…”

“…Also BD was shown to significantly improve with azacytidine . In 2 following reports describing 3 patients each, different autoimmune conditions were reported to respond to this hypomethylating agent, such as Sweet's syndrome, polyarthritis, and vasculitis as well as rheumatoid arthritis and fluctuating lupus‐like symptoms . In most of these patients, the autoimmune conditions were resistant to several lines of immunosuppressive therapy, and their improvement was usually paralleled by the haematological response to azacytidine.…”