Trisomy 8 Involved in Myelodysplastic Syndromes as a Risk Factor for Intestinal Ulcers and Thrombosis — Behçet's Syndrome

Abstract

Only 12 myelodysplastic syndrome (MDS) cases with Behçet's syndrome have been previously reported and trisomy 8 was found to have accumulated in all these patients. Five of the cases had complications in the form of multiple intestinal ulcers, which is one of the symptoms of Behçet's syndrome. To investigate the relationship between trisomy 8 and multiple intestinal ulcers in MDS patients, we analyzed 46 MDS cases treated in our hospital over the last decade, and trisomy 8 was observed in eight of them. Three of these cases had complications of both multiple intestinal ulcers and thrombosis, and two cases showed episodes of thrombosis without intestinal ulcers. All these five cases featured trisomy 8, while the other 38 MDS patients without trisomy 8 had no episode of either intestinal ulcer or thrombosis. Two of the three cases suffering from multiple intestinal ulcers were treated with granulocyte-colony stimulating factor (G-CSF), which resulted in aggravation of the symptoms. Although the influence of G-CSF on such symptoms in MDS patients with trisomy 8 remains unclear, it seems advisable to exercise caution in the use of G-CSF when an MDS patient with trisomy 8 has intestinal ulcers or thrombosis.

Simultaneous Occurrence of Inflammatory Bowel Disease and Myelodysplastic Syndrome due to Chromosomal Abnormalities in Bone Marrow Cells

“…He was diagnosed with MDS. Trisomy 8 seen in his BM cells is a risk factor for intestinal Behçet’s disease [7,8,9]. However, endoscopic findings of this case were not consistent with those of Behçet’s disease since no round ulcerations were seen [11].…”

“…Most cases of intestinal Behçet’s disease associated with MDS have trisomy 8 as a chromosomal abnormality [7,8,9]. However, intestinal Behçet’s disease is not so common in MDS patients harboring trisomy 8, suggesting that this chromosomal abnormality is not enough to cause IBD.…”

“…Given that the dysregulation of BM-derived APCs is involved in the development of IBD, it is possible that BM cells with chromosomal abnormalities cause both IBD and MDS. In fact, trisomy 8, a frequent cytogenetic abnormality in MDS patients, is known as a risk factor for intestinal Behçet’s disease [7,8,9]. Moreover, several cases with a simultaneous occurrence of IBD and MDS are reported [10].…”

Myelodysplastic Syndrome Complicated with Inflammatory Intestinal Ulcers: Significance of Trisomy 8

“…In Case 2, each attack was accompanied by an elevated serum CK level, suggesting the presence of myositis in the acute phase (Fig. 2) (12). Excessive production of inflammatory cytokines and abnormal neutrophil functions are frequently observed in MDS (22,23 (25).…”

Successful Treatment of Myelodysplastic Syndrome (MDS)-related intestinal Behcet's Disease by Up-front Cord Blood Transplantation

“…Interestingly, it is speculated that this is not a simple coexistence but that the aetiology of intestinal Behçet's disease is at least partly derived from MDS itself (2,5,11,12 (4,6,9 CT s c a n s s h o we d ma r k e d e d e ma o f t h e i l e o c e c u m r e g i o n ( a r r o ws ) a n d i n f l a mma t i o n o f t h e s u r r o u n d i n g f a t t i s s u e . dup (1) …”