title: Periodic fever and erythema nodosum associated with MDS with trisomy 8: report of two cases and review of the literature.
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.
authors.affiliation: Department of Rheumatology and Clinical Immunology, Kyoto University Hospital, Sakyo-ku, Kyoto, Japan.
authors.forename: Takanori
authors.initials: T
authors.lastname: Fujimura
authors.forename: Naoichiro
authors.initials: N
authors.lastname: Yukawa
authors.forename: Ran
authors.initials: R
authors.lastname: Nakashima
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Imura
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kawabata
authors.forename: Takaki
authors.initials: T
authors.lastname: Nojima
authors.forename: Koichiro
authors.initials: K
authors.lastname: Ohmura
authors.forename: Takao
authors.initials: T
authors.lastname: Fujii
authors.forename: Takashi
authors.initials: T
authors.lastname: Usui
authors.forename: Tsuneyo
authors.initials: T
authors.lastname: Mimori
mesh_terms: D000368:Aged; D001528:Behcet Syndrome / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D002898:Chromosomes, Human, Pair 8; D003937:Diagnosis, Differential; D004893:Erythema Nodosum / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D005334:Fever / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D014314:Trisomy