The association of Behçet`s disease with myelodysplastic syndrome in Japan: A review of the literature

CER2857
2006 Vol.24, N°5 ,Suppl.42
PI 0115, PF 0119
Review

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PMID: 17067441 [PubMed]

Abstract

OBJEVTIVE: To determine the clinical characteristics of patients with myelodysplastic syndrome (MDS)-associated Behçet`s disease (BD) in Japan.
METHODS:
54 Japanese cases of MDS-associated BD obtained from the literature and from our own clinical experience were reviewed. The clinical features of MDS-associated BD were compared with those of the 1991 nationwide BD survey in Japan.
RESULTS:
In MDS-associated BD, the average age at onset was 42.6 years, which was 6.9 years later than for all BD patients; females developed disease more frequently than males (male: female ratio = 0.80). In MDS-associated BD cases, the occurrence of eye lesions was significantly lower, the frequency of intestinal lesions was markedly higher, and the rate of HLA-B51 positivity was lower than that in all BD. BD and MDS developed nearly simultaneously in 49.0% of cases; BD preceded MDS in 31.4% of the cases. The distribution of the age at BD onset showed two peaks, one in the 3^rd decade and the other in the 6^th decade. Females were more likely to develop younger-onset disease, while men were more likely to develop older-onset MDS-associated BD. Furthermore, in the older-onset group, BD was diagnosed together with or after the diagnosis of MDS, while half of the younger-onset group developed BD earlier than MDS.
CONCLUSIONS:
MDS-associated BD patients form a distinct subset of patients. There may, in fact, be two major groups of MDS-associated BD patients based on age, gender, and temporal relationship of the two diseases.