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Long‐term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single‐center retrospective study
Author(s) -
Sulpizio Emilio D.,
Raghunathan Vikram,
Shatzel Joseph J.,
ZilbermanRudenko Jevgenia,
Worrest Tarin,
Sheppard Brett C.,
DeLoughery Thomas G.
Publication year - 2020
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.13336
Subject(s) - evans syndrome , splenectomy , medicine , autoimmune hemolytic anemia , retrospective cohort study , cohort , thrombocytopenic purpura , surgery , anemia , pediatrics , spleen , platelet
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.