Abstract:
:Immune dysregulation and altered T-cell hemostasis play important roles in the pathogenesis of myelodysplastic syndromes (MDS). Recent studies suggest an increased risk of MDS among patients with autoimmune diseases. Here, we investigated the prevalence of autoimmune diseases among MDS patients, comparing characteristics and outcomes in those with and without autoimmune diseases. From our study group of 1408 MDS patients, 391 (28%) had autoimmune disease, with hypothyroidism being the most common type, accounting for 44% (n = 171) of patients (12% among all MDS patients analyzed). Other autoimmune diseases with ≥5% prevalence included idiopathic thrombocytopenic purpura in 12% (n = 46), rheumatoid arthritis in 10% (n = 41), and psoriasis in 7% (n = 28) of patients. Autoimmune diseases were more common in female MDS patients, those with RA or RCMD WHO subtype, and those who were less dependent on red blood cell transfusion. Median overall survival (OS) was 60 months (95% CI, 50-70) for patients with autoimmune diseases versus 45 months (95% CI, 40-49) for those without (log-rank test, P = 0.006). By multivariate analysis adjusting for revised IPSS and age >60 years, autoimmune diseases were a statistically significant independent factor for OS (HR 0.78; 95% CI, 0.66-0.92; P = 0.004). The rate of acute myeloid leukemia (AML) transformation was 23% (n = 89) in MDS patients with autoimmune disease versus 30% (n = 301) in those without (P = 0.011). Patient groups did not differ in response to azacitidine or lenalidomide treatment. Autoimmune diseases are prevalent among MDS patients. MDS patients with autoimmune diseases have better OS and less AML transformation.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Komrokji RS,Kulasekararaj A,Al Ali NH,Kordasti S,Bart-Smith E,Craig BM,Padron E,Zhang L,Lancet JE,Pinilla-Ibarz J,List AF,Mufti GJ,Epling-Burnette PKdoi
10.1002/ajh.24333subject
Has Abstractpub_date
2016-05-01 00:00:00pages
E280-3issue
5eissn
0361-8609issn
1096-8652journal_volume
91pub_type
杂志文章abstract::Serum lactic dehydrogenase (LDH) levels are mildly elevated in beta-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients h...
journal_title:American journal of hematology
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doi:10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13
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pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2017-09-01 00:00:00
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更新日期:2014-06-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199803)57:3<253::aid-ajh14
更新日期:1998-03-01 00:00:00
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journal_title:American journal of hematology
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pub_type: 杂志文章
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journal_title:American journal of hematology
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更新日期:1998-04-01 00:00:00
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journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
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更新日期:1996-02-01 00:00:00
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journal_title:American journal of hematology
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pub_type: 杂志文章,多中心研究
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journal_title:American journal of hematology
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更新日期:1994-08-01 00:00:00
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