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

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Modern Rheumatology

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.

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Acknowledgments

We thank Dr. Hiroshi Kawabata, Department of Hematology and Oncology, Kyoto University Hospital for the generous suggestion of the patients.

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Authors and Affiliations

  1. Department of Rheumatology and Clinical Immunology, Kyoto University Hospital, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan

    Takanori Fujimura, Naoichiro Yukawa, Ran Nakashima, Daisuke Kawabata, Takaki Nojima, Koichiro Ohmura, Takao Fujii, Takashi Usui & Tsuneyo Mimori

  2. Department of Rheumatology, Osaka Red Cross Hospital, Osaka, Japan

    Yoshitaka Imura

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  1. Takanori Fujimura
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  2. Naoichiro Yukawa
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  3. Ran Nakashima
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Correspondence to Naoichiro Yukawa.

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Fujimura, T., Yukawa, N., Nakashima, R. et al. Periodic fever and erythema nodosum associated with MDS with trisomy 8: report of two cases and review of the literature. Mod Rheumatol 20, 413–419 (2010). https://doi.org/10.1007/s10165-010-0291-9

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  • DOI: https://doi.org/10.1007/s10165-010-0291-9

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