Background: Immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) show good responses to frontline steroids. About two-third of cases relapse and require second-line treatment, including rituximab, mainly effective in AIHA, and thrombopoietin-receptor agonists (TPO-RAs) in ITP, while the use of splenectomy progressively decreased due to concerns for infectious/thrombotic complications. For those failing second line, immunosuppressants may be considered.Objectives: The aim of this study was to evaluate the efficacy of cyclosporine treatment in patients with ITP and AIHA.Design: In this retrospective study, we evaluated the efficacy and safety of cyclosporine A (CyA) in ITP (N=29) and AIHA (N=10) patients followed at two reference centers in Milan, Italy.Methods: Responses were classified as partial [Hb >10 or at least 2 g/dl increase from baseline, platelets (PLT) > 30 x 10(9)/l with at least doubling from baseline] and complete (Hb > 12 g/dl or PLT > 100 x 10(9)/l) and evaluated at 3,6, and 12 months. Treatment emergent adverse events were also registered.Results: The median time from diagnosis to CyA was 35 months (3-293), and patients had required a median of 4 (1-8) previous therapy lines. Median duration of CyA was 28 (2-140) months and responses were achieved in 86% of ITP and 50% of AIHA subjects. Responders could reduce or discontinue concomitant treatment and resolved PLT fluctuations on TPO-RA. CyA was generally well tolerated, and only two serious infectious complications in elderly patients on concomitant steroids suggesting caution in this patient population.Conclusion: CyA may be advisable in ITP, which is not well controlled under TPO-RA, and in AIHA failing rituximab, particularly if ineligible in clinical trial.

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers / B. Fattizzo, S. Cantoni, J.A. Giannotta, L. Bandiera, R. Zavaglia, M. Bortolotti, W. Barcellini. - In: THERAPEUTIC ADVANCES IN HEMATOLOGY. - ISSN 2040-6207. - 13:(2022 Dec), pp. 20406207221097780.1-20406207221097780.11. [10.1177/20406207221097780]

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

B. Fattizzo
Primo
;
J.A. Giannotta;M. Bortolotti;
2022

Abstract

Background: Immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) show good responses to frontline steroids. About two-third of cases relapse and require second-line treatment, including rituximab, mainly effective in AIHA, and thrombopoietin-receptor agonists (TPO-RAs) in ITP, while the use of splenectomy progressively decreased due to concerns for infectious/thrombotic complications. For those failing second line, immunosuppressants may be considered.Objectives: The aim of this study was to evaluate the efficacy of cyclosporine treatment in patients with ITP and AIHA.Design: In this retrospective study, we evaluated the efficacy and safety of cyclosporine A (CyA) in ITP (N=29) and AIHA (N=10) patients followed at two reference centers in Milan, Italy.Methods: Responses were classified as partial [Hb >10 or at least 2 g/dl increase from baseline, platelets (PLT) > 30 x 10(9)/l with at least doubling from baseline] and complete (Hb > 12 g/dl or PLT > 100 x 10(9)/l) and evaluated at 3,6, and 12 months. Treatment emergent adverse events were also registered.Results: The median time from diagnosis to CyA was 35 months (3-293), and patients had required a median of 4 (1-8) previous therapy lines. Median duration of CyA was 28 (2-140) months and responses were achieved in 86% of ITP and 50% of AIHA subjects. Responders could reduce or discontinue concomitant treatment and resolved PLT fluctuations on TPO-RA. CyA was generally well tolerated, and only two serious infectious complications in elderly patients on concomitant steroids suggesting caution in this patient population.Conclusion: CyA may be advisable in ITP, which is not well controlled under TPO-RA, and in AIHA failing rituximab, particularly if ineligible in clinical trial.
Evans syndrome; autoimmune hemolytic anemia; cyclosporine A; immune thrombocytopenia
Settore MED/15 - Malattie del Sangue
14-mag-2022
Article (author)
File in questo prodotto:
File Dimensione Formato  
20406207221097780.pdf

accesso aperto

Tipologia: Publisher's version/PDF
Dimensione 175.55 kB
Formato Adobe PDF
175.55 kB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/947712
Citazioni
  • ???jsp.display-item.citation.pmc??? 3
  • Scopus 4
  • ???jsp.display-item.citation.isi??? 3
social impact