title: Probability of remaining in unsustained complete remission after steroid therapy withdrawal in patients with primary warm-antibody reactive autoimmune hemolytic anemia.
abstract: BACKGROUND
  Primary warm autoimmune hemolytic anemia (WAIHA) is a rare autoimmune disorder frequently responding to corticosteroid first-line treatment and effective second-line treatment options such as splenectomy or anti-CD20 antibody therapy. Disease management is frequently hampered by a lack of evidence.
  
  
  METHODS
  We have investigated the probability of sustained treatment-free remission after steroid induction to facilitate clinical decision making regarding timing and necessity of second-line treatments. Response data from 31 patients with primary WAIHA initially treated with steroids were retrospectively analyzed. All patients responded by achieving a hemoglobin of at least 10 mg/dl.
  
  
  RESULTS
  After steroid tapering and final withdrawal, 9 of 30 patients remained in unsustained complete remission (CR). The probability of remaining in CR after steroid treatment only was 38.2 % (2 SD 20.6 %) at 15 months. The median remission duration was 100 + months with a range of 12 + to 163 + months. Of note, none of the remaining patients still on steroids achieved CR beyond 15 + months.
  
  
  CONCLUSIONS
  These data indicate that a considerable proportion of patients do not need further treatment and that relapses will not occur after 15 months in CR.
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Johanna
authors.initials: J
authors.lastname: Kulpa
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Cathrin
authors.initials: C
authors.lastname: Skrabs
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Ralph
authors.initials: R
authors.lastname: Simanek
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
authors.affiliation: Department of Blood Group Serology and Transfusion Medicine, Medical University of Vienna, Vienna, Austria.
authors.forename: Simon
authors.initials: S
authors.lastname: Panzer
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Klaus
authors.initials: K
authors.lastname: Lechner
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Sillaber
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria. ulrich.jaeger@meduniwien.ac.at.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jäger
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology*; D001317:Austria / Q000453:epidemiology; D003198:Computer Simulation; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D015995:Prevalence; D011379:Prognosis; D012074:Remission Induction / Q000379:methods*; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Warm autoimmune hemolytic anemia; long term remission; steroids