Usefulness of Adalimumab for Treating a Case of Intestinal Behcet's Disease With Trisomy 8 Myelodysplastic Syndrome.
- Author:
Masamichi KIMURA
1
;
Yoshihisa TSUJI
;
Masako IWAI
;
Masahiro INAGAKI
;
Ali MADIAN
;
Takuya YOSHINO
;
Minoru MATSUURA
;
Hiroshi NAKASE
Author Information
- Publication Type:Case Report
- Keywords: Adalimumab; Behcet syndrome; Trisomy 8; Myelodysplastic syndromes
- MeSH: Adalimumab; Arthritis, Rheumatoid; Autoimmune Diseases; Behcet Syndrome; Bone Marrow Cells; Cell Death; Hematopoiesis; Humans; Inflammatory Bowel Diseases; Myelodysplastic Syndromes*; Systemic Vasculitis; Trisomy*; Tumor Necrosis Factor-alpha
- From:Intestinal Research 2015;13(2):166-169
- CountryRepublic of Korea
- Language:English
- Abstract: Behcet's disease (BD) is a systemic vasculitis, while myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematologic disorders characterized by ineffective hematopoiesis. Some studies suggest a relationship between MDS and BD, especially intestinal BD, and trisomy 8 seems to play an important role in both diseases. There are several reports on patients with BD comorbid with MDS involving trisomy 8 that frequently have intestinal lesions refractory to conventional medical therapies. Tumor necrosis factor (TNF)-alpha is strongly involved in the pathophysiology of several autoimmune diseases such as rheumatoid arthritis, inflammatory bowel disease, and BD. In addition, TNF-alpha plays an important role in the pathophysiology of MDS by inhibiting normal hematopoiesis and inducing the programmed cell death of normal total bone marrow cells and normal CD34+ cells. Recent clinical reports demonstrate the favorable effect of TNF-alpha antagonists in patients with refractory intestinal BD and in those with MDS. We present the case of a patient with intestinal BD and MDS involving trisomy 8 who was successfully treated with adalimumab.
