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KMID : 0988920150130020166
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Intestinal Research
2015 Volume.13 No. 2 p.166 ~ p.169
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Usefulness of Adalimumab for Treating a Case of Intestinal Behcet’s Disease With Trisomy 8 Myelodysplastic Syndrome
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Kimura Masamichi
Tsuji Yoshihisa
Iwai Masako
Inagaki Masahiro
Madian Ali
Yoshino Takuya
Matsuura Minoru
Nakase Hiroshi
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Abstract
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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)-αis strongly involved in the pathophysiology of several autoimmune diseases such as rheumatoid arthritis, inflammatory bowel disease, and BD. In addition, TNF-α 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-α 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. (Intest Res 2015;13:166-169)
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KEYWORD
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Adalimumab, Behcet syndrome, Trisomy 8, Myelodysplastic syndromes
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