A case of Behçet’s disease complicated with myelodysplastic syndrome |
Kyung Hoon Kim, Ji Min Kim, Seung Woo Han, Eon Jeong Nam, Young Mo Kang |
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증례 : 베체트병에 동반된 골수형성이상 증후군 1예 |
김경훈.김지민.한승우.남언정.강영모, Ji Min Kim, Seung Woo Han, Eon Jeong Nam, Young Mo Kang |
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Abstract |
Behçet’s disease (BD) is a chronic systemic inflammatory vasculitis. Its pathogenesis includes neutrophil hyperfunction and the overproduction of inflammatory cytokines, including TNF-α. BD is often accompanied by leukocytosis, but is rarely associated with myelodysplastic syndrome (MDS). MDS is an acquired clonal hematopoietic stem cell disorder, characterized by ineffective, dysplastic hematopoiesis with peripheral cytopenia. The development of MDS is associated with genetic abnormalities, abnormal microenvironmental and immunological influences, and drugs. A few cases of BD complicated with MDS have been reported, many of which have intestinal ulcers, refractory anemia (RA), and a cytogenetic abnormality of trisomy 8. We present a case of BD complicated with MDS, which had intestinal ulcers unresponsive to immunosuppressive agents, and a subtype of RA with the double karyotypic abnormalities 1q duplication and Y deletion, instead of trisomy 8. (Korean J Med 75:S921-S925, 2008) |
Key Words:
Behçet’s disease; Myelodysplastic syndrome |
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