Vol.20 No.4

Case Report

Periodic fever and erythema nodosum associated with MDS with trisomy 8: report of two cases and review of the literature

Authors

Takanori Fujimura¹ , Naoichiro Yukawa¹ , Ran Nakashima¹ , Yoshitaka Imura² , Daisuke Kawabata¹ , Takaki Nojima¹ , Koichiro Ohmura¹ , Takao Fujii¹ , Takashi Usui¹ , Tsuneyo Mimori¹

Received:

7 January 2010

Accepted:

25 February 2010

Published online:

8 April 2010

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Abstract

We report two cases of myelodysplastic syndrome (MDS) with trisomy 8 who had periodic fever and erythema nodosum (EN). A 74-year-old man showed periodic fever and EN. A diagnosis of MDS with trisomy 8 was made, and he was successfully treated with prednisolone (PSL). A 71-year-old man presented with intermittent fever, EN, and recurrent elevation of myogenic enzymes. Despite sustained inflammation, laboratory tests showed macrocytic anemia and thrombocytopenia. Marrow aspiration showed MDS with the chromosomal abnormality trisomy 8. He was successfully treated with PSL without repeated transient fever and elevation of creatine kinase. The results of a literature review of 35 cases of MDS with trisomy 8 and Behçet’s disease-like symptoms, such as EN, oral ulcer and intestinal ulcer, suggest that the disease entity of “trisomy 8 syndrome” may be considered, and that it is an important differential diagnosis of periodic fever and EN.

Key words

Behçet’s disease - Erythema nodosum - Fever - Myelodysplastic syndrome - Trisomy