Skip to main content
Erschienen in: Wiener klinische Wochenschrift 7-8/2016

01.04.2016 | original article

Probability of remaining in unsustained complete remission after steroid therapy withdrawal in patients with primary warm-antibody reactive autoimmune hemolytic anemia

verfasst von: Johanna Kulpa, Cathrin Skrabs, Ralph Simanek, Peter Valent, Simon Panzer, Klaus Lechner, Christian Sillaber, Ulrich Jäger, MD

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 7-8/2016

Einloggen, um Zugang zu erhalten

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.
Literatur
1.
Zurück zum Zitat Lechner K, Jäger U, How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831–8.CrossRefPubMed Lechner K, Jäger U, How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831–8.CrossRefPubMed
2.
Zurück zum Zitat 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). 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).
3.
Zurück zum Zitat Murphy S, LoBuglio AF. Drug therapy of autoimmune hemolytic anemia. Semin Hematol. 1976;13(4):323–34.PubMed Murphy S, LoBuglio AF. Drug therapy of autoimmune hemolytic anemia. Semin Hematol. 1976;13(4):323–34.PubMed
4.
Zurück zum Zitat 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.CrossRefPubMed 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.CrossRefPubMed
6.
Zurück zum Zitat 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.CrossRefPubMed 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.CrossRefPubMed
8.
Zurück zum Zitat 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.CrossRefPubMed 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.CrossRefPubMed
9.
Zurück zum Zitat 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.PubMed 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.PubMed
10.
Zurück zum Zitat 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.CrossRefPubMed 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.CrossRefPubMed
Metadaten
Titel
Probability of remaining in unsustained complete remission after steroid therapy withdrawal in patients with primary warm-antibody reactive autoimmune hemolytic anemia
verfasst von
Johanna Kulpa
Cathrin Skrabs
Ralph Simanek
Peter Valent
Simon Panzer
Klaus Lechner
Christian Sillaber
Ulrich Jäger, MD
Publikationsdatum
01.04.2016
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 7-8/2016
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-015-0863-y

Weitere Artikel der Ausgabe 7-8/2016

Wiener klinische Wochenschrift 7-8/2016 Zur Ausgabe

MUW researcher of the month

Researcher of the Month Thomas Pezawas