Intest Res.  2015 Apr;13(2):166-169. 10.5217/ir.2015.13.2.166.

Usefulness of Adalimumab for Treating a Case of Intestinal Behcet's Disease With Trisomy 8 Myelodysplastic Syndrome

Affiliations
  • 1Department of Gastroenterology and Hepatology, Kyoto University Hospital, Kyoto, Japan. ytsuji@kuhp.kyoto-u.ac.jp
  • 2Department of Internal Medicine, Al Azhar University, Assint, Egypt.

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.

Keyword

Adalimumab; Behcet syndrome; Trisomy 8; Myelodysplastic syndromes

MeSH Terms

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
Tumor Necrosis Factor-alpha

Figure

  • Fig. 1 Endoscopic findings before and after adalimumab (ADA) treatment. (A and B) Endoscopic findings prior to ADA treatment revealing redness, edematous mucosa, and multiple punched-out ulcers located at the postoperative anastomosis and the ileum. (C and D) Endoscopic findings showing marked improvement of the inflamed mucosa 1 year after starting ADA treatment.


Cited by  1 articles

Advances in Management of Intestinal Behçet’s Disease: A Perspective From Gastroenterologists
Jae Hee Cheon
J Rheum Dis. 2021;28(1):4-16.    doi: 10.4078/jrd.2021.28.1.4.


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