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LONG TERM SURVIVAL OF PATIENTS WITH EVANS SYNDROME: A POPULATION-BASED COHORT STUDY
Author(s): ,
Dennis Lund Hansen
Affiliations:
Department of Haematology,Odense University Hospital,Odense,Denmark
,
Sören Möller
Affiliations:
OPEN,Odense University Hospital and University of Southern Denmark,Odense,Denmark
,
Nikolaj Mannering
Affiliations:
Department of Haematology,Odense University Hospital,Odense,Denmark
,
Kjeld Andersen
Affiliations:
Department of Psychiatry ,Region of Southern Denmark,Odense,Denmark
,
David Gaist
Affiliations:
Department of Neurology,Odense University Hospital,Odense,Denmark
Henrik Frederiksen
Affiliations:
Department of Haematology,Odense University Hospital,Odense,Denmark
(Abstract release date: 05/17/18) EHA Library. Lund Hansen D. 06/15/18; 214601; S146
Ms. Dennis Lund Hansen
Ms. Dennis Lund Hansen
Contributions
Abstract

Abstract: S146

Type: Oral Presentation

Presentation during EHA23: On Friday, June 15, 2018 from 12:00 - 12:15

Location: Room A10

Background
Evans syndrome (ES) is defined by either simultaneous or sequential diagnoses of both immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA).  The syndrome is rare, and data on frequency and prognosis are lacking. Earlier reports have focused mainly on complications and relapse (e.g.: Michel, Blood. 2009, Costallat, Joint Bone Spine. 2012). Here we report novel epidemiological data on primary and secondary ES from a nationwide register.

Aims
To provide population-based estimates of incidence, prevalence and survival of adults with ES.

Methods
To estimate these parameters we followed a nationwide cohort of patients with at least one diagnosis of ITP or haemolysis according to the Danish National Patient Register, January 1977 to December 2015. We excluded patients less than thirteen years of age, due to the usually self-limiting nature of childhood ITP. A comparison cohort comprised individuals from the general population matched to the patient cohort (50:1) on age, and gender.

The patient cohort was classified as either primary or secondary ES based on the following diagnoses (recorded at any time before or up to one year after date of ES diagnosis):  hereditary and non-familial hypogammaglobulinemia, systemic lupus erythematosus (SLE), HIV/AIDS, hepatitis C, liver cirrhosis, autoimmune lymphoproliferative syndrome type I and haematological malignancies including myelofibrosis.

Results
The patient cohort included 25,238 patients with a haemolysis diagnosis or ITP. From this cohort we identified 209 patients with ES aged 13-97 years. Simultaneous diagnoses were present in 32.5%, and ITP was the first symptom in 44.5% of the patients. Mean time between the two diagnoses was 3 years [95%CI: 2.2-3.7]. The patients with ES made up 2% [95%CI: 1.8-2.3] of all ITP cases, and 8% [95%CI: 7-9] of the AIHA patients.

54.1% [95%CI: 46.0-62.0] of the primary ES patients, and 46.0% [95%CI: 31.8-60.7] of the secondary cases were women.

Age at diagnosis was 57.3 years [95%CI: 54.1-60.6] in the primary ES group and 62.6 [95%CI: 57.6-67.6] years in the secondary ES group.

23.9% [95%CI: 18.3-30.3] were secondary ES, with haematological malignancy as the predominant cause (68.0%).

The five-year cumulated incidence of ES (primary or secondary) in number of new cases per 1 million inhabitants, was 6.1 [95%CI: 3.8-9.4] in 1981-1985; 4.5 [95%CI: 2.6-7.3] in 1996-2000; and 12.6 [95%CI: 9.3-16.7] in 2011-2015.

The prevalence in number of cases per 1 million inhabitants was in 1985 5.1 [95%CI: 3.2-7.7]; increasing to 8.7 [95%CI: 6.2-11.9] in 2000; and 18.2 [95%CI: 14.6-22.4] in 2015.

Age at death was 64.6 years [95%CI: 61.7-67.4] in patients with primary ES and 65.9 years [95%CI: 61.3-70.5] in those with secondary ES. Median survival was 7.9 years [95%CI: 5.5-11.7] in primary ES, and 2.2 years [95%CI: 1.1-10.4] in secondary ES, compared to 21.6 years [95%CI: 20.9-22.4] in the comparison cohort from the general population (Figure). Median survival was stable during the period.

Conclusion
ES is associated with a significant increase in mortality, especially among secondary cases. Incidence and prevalence are increasing, but survival does not seem to improve.

Session topic: 29. Enzymopathies, membranopathies and other anemias

Keyword(s): Autoimmune hemolytic anemia (AIHA), epidemiology, Immune thrombocytopenia (ITP), Survival

Abstract: S146

Type: Oral Presentation

Presentation during EHA23: On Friday, June 15, 2018 from 12:00 - 12:15

Location: Room A10

Background
Evans syndrome (ES) is defined by either simultaneous or sequential diagnoses of both immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA).  The syndrome is rare, and data on frequency and prognosis are lacking. Earlier reports have focused mainly on complications and relapse (e.g.: Michel, Blood. 2009, Costallat, Joint Bone Spine. 2012). Here we report novel epidemiological data on primary and secondary ES from a nationwide register.

Aims
To provide population-based estimates of incidence, prevalence and survival of adults with ES.

Methods
To estimate these parameters we followed a nationwide cohort of patients with at least one diagnosis of ITP or haemolysis according to the Danish National Patient Register, January 1977 to December 2015. We excluded patients less than thirteen years of age, due to the usually self-limiting nature of childhood ITP. A comparison cohort comprised individuals from the general population matched to the patient cohort (50:1) on age, and gender.

The patient cohort was classified as either primary or secondary ES based on the following diagnoses (recorded at any time before or up to one year after date of ES diagnosis):  hereditary and non-familial hypogammaglobulinemia, systemic lupus erythematosus (SLE), HIV/AIDS, hepatitis C, liver cirrhosis, autoimmune lymphoproliferative syndrome type I and haematological malignancies including myelofibrosis.

Results
The patient cohort included 25,238 patients with a haemolysis diagnosis or ITP. From this cohort we identified 209 patients with ES aged 13-97 years. Simultaneous diagnoses were present in 32.5%, and ITP was the first symptom in 44.5% of the patients. Mean time between the two diagnoses was 3 years [95%CI: 2.2-3.7]. The patients with ES made up 2% [95%CI: 1.8-2.3] of all ITP cases, and 8% [95%CI: 7-9] of the AIHA patients.

54.1% [95%CI: 46.0-62.0] of the primary ES patients, and 46.0% [95%CI: 31.8-60.7] of the secondary cases were women.

Age at diagnosis was 57.3 years [95%CI: 54.1-60.6] in the primary ES group and 62.6 [95%CI: 57.6-67.6] years in the secondary ES group.

23.9% [95%CI: 18.3-30.3] were secondary ES, with haematological malignancy as the predominant cause (68.0%).

The five-year cumulated incidence of ES (primary or secondary) in number of new cases per 1 million inhabitants, was 6.1 [95%CI: 3.8-9.4] in 1981-1985; 4.5 [95%CI: 2.6-7.3] in 1996-2000; and 12.6 [95%CI: 9.3-16.7] in 2011-2015.

The prevalence in number of cases per 1 million inhabitants was in 1985 5.1 [95%CI: 3.2-7.7]; increasing to 8.7 [95%CI: 6.2-11.9] in 2000; and 18.2 [95%CI: 14.6-22.4] in 2015.

Age at death was 64.6 years [95%CI: 61.7-67.4] in patients with primary ES and 65.9 years [95%CI: 61.3-70.5] in those with secondary ES. Median survival was 7.9 years [95%CI: 5.5-11.7] in primary ES, and 2.2 years [95%CI: 1.1-10.4] in secondary ES, compared to 21.6 years [95%CI: 20.9-22.4] in the comparison cohort from the general population (Figure). Median survival was stable during the period.

Conclusion
ES is associated with a significant increase in mortality, especially among secondary cases. Incidence and prevalence are increasing, but survival does not seem to improve.

Session topic: 29. Enzymopathies, membranopathies and other anemias

Keyword(s): Autoimmune hemolytic anemia (AIHA), epidemiology, Immune thrombocytopenia (ITP), Survival

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