Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comparative Study
. 2020 Jan;104(1):55-58.
doi: 10.1111/ejh.13336. Epub 2019 Oct 27.

Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study

Emilio D Sulpizio  1 , Vikram Raghunathan  2 , Joseph J Shatzel  2 , Jevgenia Zilberman-Rudenko  3 , Tarin Worrest  4 , Brett C Sheppard  4 , Thomas G DeLoughery  2
Affiliations
Comparative Study

Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study

Emilio D Sulpizio et al. Eur J Haematol. 2020 Jan.

Abstract

Objective: Evans syndrome, the combination of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) or autoimmune neutropenia, is associated with a high rate of relapsed/refractory disease. There are limited data on the efficacy of splenectomy for this condition. We reviewed patient outcomes after splenectomy for Evans syndrome compared to ITP at our institution.

Methods: We performed a retrospective analysis of patients who underwent splenectomy for autoimmune cytopenias over a 23-year period with the intention of comparing disease relapse rates after splenectomy in patients with Evans syndrome and in those with ITP.

Results: During the study period, 77 patients underwent splenectomy for ITP and seven underwent splenectomy for Evans syndrome. In the Evans cohort, splenectomy led to an 85.7% initial response rate with a 42.8% rate of relapse within one year and a long-term (one-year) response rate of 42.8%. In the ITP cohort, the initial response rate was 90.9% with a long-term response rate of 70.1%.

Conclusion: Our data suggest that long-term remission rates after splenectomy are lower in adults with Evans syndrome compared to those with ITP, although splenectomy may still be an acceptable treatment for certain patients with Evans syndrome. Our findings underscore the need for further research and development of additional therapeutic strategies for this patient population.

Keywords: red cell disorders; thrombocytes.

PubMed Disclaimer

Conflict of interest statement

CONFLICT OF INTEREST

The authors have nothing to disclose.

References

    1. Miano M How i manage Evans syndrome and AIHA cases in children. Br J Haematol. 2016;172(4):524–534. - PubMed
    1. Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167–3172. - PubMed
    1. Lechner K, Jäger U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831–1838. - PubMed
    1. Lambert MP, Gernsheimer TB. Clinical updates in adult immune thrombocytopenia. Blood. 2017;129(21):2829–2835. - PMC - PubMed
    1. Ahmed R, Devasia AJ, Viswabandya A, et al. Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children: splenectomy in ITP. Ann Hematol. 2016;95(9):1429–1434. - PubMed

Publication types

Supplementary concepts