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2016 ; 95
(13
): e3091
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Certain Autoimmune Manifestations Are Associated With Distinctive Karyotypes and
Outcomes in Patients With Myelodysplastic Syndrome: A Retrospective Cohort Study
#MMPMID27043672
Lee SJ
; Park JK
; Lee EY
; Joo SH
; Jung KC
; Lee EB
; Song YW
; Yoon SS
Medicine (Baltimore)
2016[Mar]; 95
(13
): e3091
PMID27043672
show ga
Autoimmune manifestations (AIMs) are common in patients with myelodysplastic
syndrome (MDS). This study aimed to investigate whether AIMs are associated with
a specific cytogenetic abnormalities and worse survival in patients with MDS. A
total of 67 MDS patients with AIMs and 134 age- and sex-matched MDS patients
without AIMs, all of whom received medical care at Seoul National University
Hospital from January 2000 through July 2014, were enrolled. The clinical
features, chromosomal abnormalities, and outcomes were examined. The effect of
AIMs on mortality was estimated after adjusting for age, sex, and the
International Prognostic Scoring System. The mean age (±SD) at the time of MDS
diagnosis was 54.5?±?17.1 years, and 44.8% of patients were male. Neutrophilic
dermatosis (ND; Sweet syndrome and pyoderma gangrenosum) was the most prevalent
AIM (n?=?24 36%]), followed by Behcet disease (10 [15%]), rheumatoid arthritis (9
[13%]), vasculitis (8 [12%]), myositis (3 [4%]), spondyloarthropathy (3 [4%]),
and systemic lupus erythematous (2 [3%]). ND and vasculitis occurred at the time
of MDS diagnosis, whereas other AIMs occurred years after MDS diagnosis. Deletion
of 5q was associated with ND (P?=?0.001), whereas trisomy 8 was associated with
Behcet disease (P?=?0.015). Strikingly, ND was associated with a 1.8-fold
increase in mortality (95% CI 1.033-3.093; P?=?0.038). Certain AIMs in MDS
patients are associated with distinctive karyotypes and worse survival. A larger
study is needed to confirm whether the presence of AIMs influences disease
outcome in MDS.