Summary
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.
Conclusion
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.
Similar content being viewed by others
References
Lechner K, Jäger U, How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831–8.
Jäger U, Lechner K. Autoimmune haemolytic anemia. In: Hoffman R, Benz EJ, Jr., Silberstein LE, Heslop HE, Weitz JI, Anastasi J, editors. Hematology—basic principles and practice, 6th ed. Chapter 44. Philadelphia: Elsevier Saunders; 2013. pp. 634–627 (ISBN 978-1-4377-2928-3).
Murphy S, LoBuglio AF. Drug therapy of autoimmune hemolytic anemia. Semin Hematol. 1976;13(4):323–34.
Allgood JW, Chaplin H, Jr. Idiopathic acquired autoimmune hemolytic anemia. A review of forty-seven cases treated from 1955 through 1965. Am J Med. 1967;43(2):254–73.
Michel M. Warm autoimmune hemolytic anemia: advances in pathophysiology and treatment. Presse Med. 2014;43(4 Pt 2):e97–104. doi:10.1016/j.lpm.2014.02.009.
Roumier M, Loustau V, Guillaud C, Languille L, Mahevas M, Khellaf M, Limal N, Noizat-Pirenne F, Godeau B, Michel M. Characteristics and outcome of warm autoimmune hemolytic anemia in adults: new insights based on a single-center experience with 60 patients. Am J Hematol. 2014;89(9):E150–5. doi:10.1002/ajh.23767.
Sinwar PD. Overwhelming post splenectomy infection syndrome - review study. Int J Surg. 2014;12(12):1314–6. doi:10.1016/j.ijsu.2014.11.005.
Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, Larsen H, Mourits-Andersen T, Plesner T, Rønnov-Jessen D, Vestergaard H, Klausen TW, Schöllkopf C. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol. 2013;163(3):393–9. doi:10.1111/bjh.12541.
Sailer T, Lechner K, Panzer S, Kyrle PA, Pabinger I. The course of severe autoimmune thrombocytopenia in patients not undergoing splenectomy. Haematologica. 2006;91(8):1041–5.
Reynaud Q, Durieu I, Dutertre M, Ledochowski S, Durupt S, Michallet A, Vital-Durand D, Lega J. Efficacy and safety of rituximab in auto-immune hemolytic anemia: a meta-analysis of 21 studies. Autoimmun Rev. 2015;14:304–13.
Acknowledgments
Expert technical assistance by Michaela Bronhagl is greatly acknowledged.
Authors’ contributions
All authors have read and approved the final version of the manuscript.
The study design was done by K. Lechner and U. Jäger. Data analysis was done by J. Kulpa, C. Skrabs, R. Simanek, P. Valent, S. Panzer, and C. Sillaber. Manuscript writing was done by K. Lechner and U. Jäger.
Conflict of interest
We hereby confirm that none of the authors has a conflict of interest with regard to the study or its results.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kulpa, J., Skrabs, C., Simanek, R. et al. Probability of remaining in unsustained complete remission after steroid therapy withdrawal in patients with primary warm-antibody reactive autoimmune hemolytic anemia. Wien Klin Wochenschr 128, 234–237 (2016). https://doi.org/10.1007/s00508-015-0863-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00508-015-0863-y