title: Autoimmune diseases and myelodysplastic syndromes.
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.
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Rami S
authors.initials: RS
authors.lastname: Komrokji
authors.affiliation: Department of Haematological Medicine, King's College Hospital, London, United Kingdom.
authors.forename: Austin
authors.initials: A
authors.lastname: Kulasekararaj
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Najla H
authors.initials: NH
authors.lastname: Al Ali
authors.affiliation: Department of Haematological Medicine, King's College Hospital, London, United Kingdom.
authors.forename: Shahram
authors.initials: S
authors.lastname: Kordasti
authors.affiliation: Department of Haematological Medicine, King's College Hospital, London, United Kingdom.
authors.forename: Emily
authors.initials: E
authors.lastname: Bart-Smith
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Benjamin M
authors.initials: BM
authors.lastname: Craig
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Eric
authors.initials: E
authors.lastname: Padron
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Ling
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Lancet
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Javier
authors.initials: J
authors.lastname: Pinilla-Ibarz
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Alan F
authors.initials: AF
authors.lastname: List
authors.affiliation: Department of Haematological Medicine, King's College Hospital, London, United Kingdom.
authors.forename: Ghulam J
authors.initials: GJ
authors.lastname: Mufti
authors.affiliation: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida.
authors.forename: Pearlie K
authors.initials: PK
authors.lastname: Epling-Burnette
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000453:epidemiology*; D001374:Azacitidine / Q000627:therapeutic use; D015897:Comorbidity; D018450:Disease Progression; D004198:Disease Susceptibility; D005260:Female; D005500:Follow-Up Studies; D050031:Hashimoto Disease / Q000453:epidemiology; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D000077269:Lenalidomide; D015470:Leukemia, Myeloid, Acute / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000453:epidemiology* / Q000276:immunology; D015995:Prevalence; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use; D013967:Thyroiditis, Autoimmune / Q000453:epidemiology; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide; D000077269:Lenalidomide; D001374:Azacitidine