title: Primary Myelodysplastic Syndromes: The Mayo Clinic Experience With 1000 Patients.
abstract: OBJECTIVE
  To share our 25 years of experience with patients with primary myelodysplastic syndromes (MDS) and to describe the natural history of the disease including presenting clinical and laboratory characteristics and long-term disease outcomes.
  
  
  METHODS
  One thousand consecutive patients with primary MDS evaluated at Mayo Clinic between January 1, 1989, and May 1, 2014, were considered. The Revised International Prognostic Scoring System and other risk models were applied for risk stratification. Separate analyses were conducted for patients diagnosed before 2005 (n=531) and after 2005 (n=469).
  
  
  RESULTS
  Eighty-five percent of patients were older than 60 years (median age, 72 years), with 69% being men. The median follow-up period was 27 months (range, 0-300 months), during which time 808 (81%) deaths and 129 (13%) leukemic transformations were documented. Median survival and leukemic transformation rates were similar in patients diagnosed before or after 2005, despite the significantly higher use of hypomethylating agents in the latter group: 33 months vs 28 months (P=.46) and 13% vs 10% (P=.92), respectively. Revised International Prognostic Scoring System risk distribution was similar in patients diagnosed before or after 2005 (P=.23): 17% were categorized as very low, 36% low, 21% intermediate, 15% high, and 11% very high risk, with a median survival of 72, 43, 24, 18, and 7 months, respectively (P<.001). We found Revised International Prognostic Scoring System cytogenetic risk categorization to be suboptimal in its performance, whereas contemporary prognostic models were broadly similar in their performance.
  
  
  CONCLUSIONS
  The poor outcome in patients with MDS does not appear to have improved over time. Current risk stratification systems for MDS are not substantially different from each other. There is a dire need for drugs that are truly disease modifying and risk models that incorporate prognostically relevant mutations.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Naseema
authors.initials: N
authors.lastname: Gangat
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Mrinal M
authors.initials: MM
authors.lastname: Patnaik
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Kebede
authors.initials: K
authors.lastname: Begna
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Taxiarchis
authors.initials: T
authors.lastname: Kourelis
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Aref
authors.initials: A
authors.lastname: Al-Kali
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Elliott
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: William J
authors.initials: WJ
authors.lastname: Hogan
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Louis
authors.initials: L
authors.lastname: Letendre
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Litzow
authors.affiliation: Division of Cytogenetics, Mayo Clinic, Rochester, MN.
authors.forename: Ryan A
authors.initials: RA
authors.lastname: Knudson
authors.affiliation: Division of Cytogenetics, Mayo Clinic, Rochester, MN.
authors.forename: Rhett P
authors.initials: RP
authors.lastname: Ketterling
authors.affiliation: Division of Hematopathology, Mayo Clinic, Rochester, MN.
authors.forename: Janice M
authors.initials: JM
authors.lastname: Hodnefield
authors.affiliation: Division of Hematopathology, Mayo Clinic, Rochester, MN.
authors.forename: Curtis A
authors.initials: CA
authors.lastname: Hanson
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Animesh D
authors.initials: AD
authors.lastname: Pardanani
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN. Electronic address: tefferi.ayalew@mayo.edu.
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000628:therapy*; D010043:Outcome and Process Assessment, Health Care; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate