Original Article

Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes

Abstract

Background:  Evans syndrome (ES) is a rare immune disorder characterized by immune thrombocytopenia and warm autoimmune hemolytic anemia (wAIHA), with the possibility of autoimmune neutropenia (AIN).  Limited data due to its rarity led us to perform a descriptive analysis of demographics, outcomes, and comorbidities.

Materials and Methods:  This study used the National Inpatient Sample database to identify Evans syndrome hospitalizations (ICD-10 code D69.41) from 2016 to 2019, with national estimates. We collected demographic data, examined outcomes, and assessed patient comorbidity. STATA Version 17 was used for data analysis.

Results: In our study of 1,255 ES hospitalizations, the mean age was  50.13 years, with a relatively even gender distribution. The racial breakdown included White (59.6%), Black (17.1%), and Hispanic (14.6%). Most had Medicare (41.8%), Medicaid (20.4%), or private insurance (33.5%). Charlson Comorbidity Index scores varied, with 36.6% scoring 3 or more. Non-elective admissions accounted for 90.2% of cases, coming from various U.S. Census Divisions, with East North Central (16.2%) and Pacific (16.1%) leading. Large urban teaching hospitals handled 84.1% of cases. Mortality was 5.5%, the mean stay was 7.8 days, with total charges averaging $114,696. Notable associations included SLE (15.63%), ITP (37%), and anemia (53.5%). Inpatient risks included AKI (22.47%) and sepsis (15.33%). Interventions included red blood cell transfusion (18.65%) and platelet transfusion (10.44%).

Conclusion: This study offers key insights into ES in hospitalized adults, emphasizing demographic trends and important associations with other conditions. More research is required to enhance our understanding of ES and enhance outcomes for those affected.

1. Michel M. Adult Evans' Syndrome. Hematol Oncol Clin North Am. 2022; 36(2):381–392.
2. Audia S, Grienay N, Mounier M, et al. Evans’ Syndrome: From Diagnosis to Treatment. J Clin Med. 2020; 9 (12): 3851.
3. Fattizzo B, Michel M, Giannotta JA, et al. Evans syndrome in adults: an observational multicenter study. Blood Adv. 2021; 5(24): 5468-5478.
4. Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, et al. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018; 9:171–184.
5. Shaikh H, Mewawalla, P. Evans syndrome. In: StatPearls [Internet].Treasure Island (FL): StatPearls Publishing; 2025 Jan.
6. 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.
7. Agency for Healthcare Research and Quality. Overview of HCUP. https://www.hcup-us.ahrq.gov/overview.jsp. Accessed 24 September, 2023.
8. Khera R, Angraal S, Couch T, et al. Adherence to methodological standards in research using the national inpatient sample. JAMA. 2017;318(20):2011-2018.
9. Sundararajan V, Henderson T, Perry C, et al. New ICD-10 version of the Charlson Comorbidity Index predicted in-hospital mortality. J Clin Epidemiol. 2004;57(12):1288-94.
10. Zainal A, Salama A, Alweis R. Immune thrombocytopenic purpura. J Community Hosp Intern Med Perspect. 2019;9(1):59-61
11. Roumier M, Loustau V, Guillaud C, et al. 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.
12.Wang W, Herrod H, Pui CH, et al. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983;15(4):381-90.
13. Hansen DL, Möller S, Andersen K, et al. Evans syndrome in adults - incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019;94(10):1081-1090.
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SectionOriginal Article(s)
Keywords
Evans Syndrome; Immune thrombocytopenia; Autoimmune hemolytic anemia; Autoimmune neutropenia; Demographics; Associations
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How to Cite
1.
Patel R, Patel M, Laxmidhar F, Patel D, Patel Z, Shaikh S, Lakhatariya K. Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes. Int J Hematol Oncol Stem Cell Res. 2025;19(2):93-100.