The association of Behçet's disease with myelodysplastic syndrome in Japan: a review of the literature.
نویسندگان
چکیده
OBJECTIVE [corrected] 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 3rd decade and the other in the 6th 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. CONCLUSION 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.
منابع مشابه
Behçet's disease, myelodysplastic syndrome, trisomy 8, gastroenterological involvement--an association.
Only a limited number of cases of Behçet's disease and hematological malignancies have been reported in the literature. We report the case of a 45 year old female patient with Behçet's disease who developed myelodysplastic syndrome, refractory anemia with excess blasts in transformation subtype, with complex chromosomal abnormalities, including excess of chromosome 8, following several years of...
متن کاملBehçet's disease-like symptoms associated with myelodysplastic syndrome with trisomy 8: a case report and review of the literature.
متن کامل
Rare association of schizophrenia and unilateral Graves’ disease with contralateral thyroid hemiagenesis in two cases of McCune-Albright syndrome
The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of Gs protein. Clinical presentation is varied and is depend...
متن کاملMyeloid Cell Leukemia-1 Gene Expression and Clinicopathological Features in Myelodysplastic Syndrome
Background and Aims: Myeloid cell leukemia-1 (Mcl-1) plays a pivotal role in the survival of hematologic and solid tumors, and is known as a substantial oncogene. Studies have demonstrated the altered expression of Mcl-1 has been linked to malignancy development and poor prognosis. In this research, we have studied the expression of Mcl-1 mRNA in myelodysplastic syndrome (MDS) patients and det...
متن کاملRange Determination of Antigen Expression in Myeloid, Erythroid and Lymphoid Cell Lineages among Patients with Myelodysplastic Syndrome
Background: Myelodysplastic syndrome is a mixed clonal disorder of bone marrow progenitor cells. Understanding the pattern of the different lineage-specific, immature, and mature markers in myelodysplastic syndrome will help in setting-up the frame of reference to diagnose. Patients and Methods: We compared 60 bone marrow samples from 30 newly-diagnosed patients with myelodysplastic syndrome ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Clinical and experimental rheumatology
دوره 24 5 Suppl 42 شماره
صفحات -
تاریخ انتشار 2006