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RELATIVE SURVIVAL REACHES A PLATEAU IN HAIRY CELL LEUKEMIA (HCL): A POPULATION-BASED STUDY ON INCIDENCE, PRIMARY TREATMENT AND SURVIVAL AMONG 1,427 PATIENTS DIAGNOSED IN THE NETHERLANDS, 1989-2014
Author(s): ,
Avinash Dinmohamed
Affiliations:
Research,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands;Public Health,Erasmus University Medical Center,Utrecht,Netherlands;Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
,
Otto Visser
Affiliations:
Registration,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands
,
Ward Posthuma
Affiliations:
Internal Medicine,Reinier de Graaf Gasthuis,Delft,Netherlands
,
Reinier Raymakers
Affiliations:
Hematology,Utrecht University Medical Center,Utrecht,Netherlands
Jeanette Doorduijn
Affiliations:
Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
(Abstract release date: 05/18/17) EHA Library. Dinmohamed A. 06/23/17; 181537; P250
Dr. Avinash Dinmohamed
Dr. Avinash Dinmohamed
Contributions
Abstract

Abstract: P250

Type: Poster Presentation

Presentation during EHA22: On Friday, June 23, 2017 from 17:15 - 18:45

Location: Poster area (Hall 7)

Background

The introduction of cladribine and pentostatin has revolutionized the management of HCL as from the late 80s. As a result of that revolution, HCL patients (pts) are rarely included in clinical trials. Population-based studies can inform on issues related to outcomes of HCL pts managed in daily practice. At present, however, population-based studies that assess patterns of incidence, treatment and survival in HCL are very scarce.

Aims
The aim of this comprehensive nationwide population-based study was to assess trends in incidence, primary treatment and survival among HCL pts diagnosed in the Netherlands.

Methods
We selected all adult (≥18 years) pts diagnosed with classic HCL in the Netherlands between 1989-2014 from the nationwide Netherlands Cancer Registry with survival follow-up through February, 2016. Age-standardized incidence rates (ASR) were calculated per 1,000,000 person-years and standardized according to the European standard population. Data on primary treatment (i.e. no therapy, chemotherapy [CT] and immumotherapy [IT]) were available for individual pts. Pts were categorized into 2 periods (1989-2000 and 2001-2014) and 3 age groups (18-59, 60-69 and ≥70 years). We calculated relative survival (RS) and the relative excess risk of mortality as measures of disease-specific survival.

Results

We included a total of 1,427 newly diagnosed HCL pts in the study (median age, 59 years; age range, 22-95 years; 77% males). The annual ASR of HCL remained quite stable over time and was 3.1 and 3.3 in the first and last period, respectively. Men had a higher overall incidence than women (5.3 v 1.3 in 2001-2014). The age-specific incidence rates for males were 5.5, 15.0 and 15.3 in 2001-2014 for the three age groups. The corresponding rates for females were 1.2, 3.1 and 5.5.
 
The application of CT increased over time for all age groups. The proportions of CT for the three age groups were 56, 51 and 34% in 1989-2000, as compared with 81, 73 and 53% in 2001-2014. The corresponding proportions for IT were 21, 13 and 17% in 1989-2000, as compared with 2, 1 and 4% in 2001-2014. Lastly, the corresponding proportions for pts who did not receive therapy were 23, 36 and 49% in 1989-2000, as compared with 17, 26 and 42% in 2001-2014.
 
Overall, when corrected for age and sex, pts diagnosed in 2001-2014 had 49% lower excess mortality during the first 10 years after HCL diagnosis, as compared with pts diagnosed in 1989-2000 (P=.005). Ten-year RS (95% confidence intervals) was impressive for pts age 18-59, namely 92% (88% - 96%) and 98% (94% - 100%; P=.176) in the first and last period, respectively (Fig 1a). Most of the significant improvement was observed in pts age ≥60. More specifically, 10-year RS for pts age 60-69 increased from 82% (71% - 92%) to 99% (89% - 106%; P=.009; Fig 1b), and for pts age ≥70 from 67% (49% - 86%) to 85% (68% - 102%; P=.366; Fig 1c) between the first and last periods. In addition, older age (P<.001), but not sex (P=.058), was associated with higher excess mortality.

Conclusion
The incidence of HCL remained stable during a 26-year period in the Netherlands. RS for pts diagnosed in the period 2001-2014 eventually reached a plateau, indicating that by then their survival is comparable to that of the general population. Survival was already excellent for younger patients throughout the entire study period. Survival improvement was most pronounced for pts age ≥60, although it was not statistically significant for pts age ≥70. This could be explained by the increased use CT over time. Population-based cancer registries are useful instruments to assess outcomes of pts rarely included in clinical trials.

Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical

Keyword(s): epidemiology, Treatment, Survival, Hairy cell leukemia

Abstract: P250

Type: Poster Presentation

Presentation during EHA22: On Friday, June 23, 2017 from 17:15 - 18:45

Location: Poster area (Hall 7)

Background

The introduction of cladribine and pentostatin has revolutionized the management of HCL as from the late 80s. As a result of that revolution, HCL patients (pts) are rarely included in clinical trials. Population-based studies can inform on issues related to outcomes of HCL pts managed in daily practice. At present, however, population-based studies that assess patterns of incidence, treatment and survival in HCL are very scarce.

Aims
The aim of this comprehensive nationwide population-based study was to assess trends in incidence, primary treatment and survival among HCL pts diagnosed in the Netherlands.

Methods
We selected all adult (≥18 years) pts diagnosed with classic HCL in the Netherlands between 1989-2014 from the nationwide Netherlands Cancer Registry with survival follow-up through February, 2016. Age-standardized incidence rates (ASR) were calculated per 1,000,000 person-years and standardized according to the European standard population. Data on primary treatment (i.e. no therapy, chemotherapy [CT] and immumotherapy [IT]) were available for individual pts. Pts were categorized into 2 periods (1989-2000 and 2001-2014) and 3 age groups (18-59, 60-69 and ≥70 years). We calculated relative survival (RS) and the relative excess risk of mortality as measures of disease-specific survival.

Results

We included a total of 1,427 newly diagnosed HCL pts in the study (median age, 59 years; age range, 22-95 years; 77% males). The annual ASR of HCL remained quite stable over time and was 3.1 and 3.3 in the first and last period, respectively. Men had a higher overall incidence than women (5.3 v 1.3 in 2001-2014). The age-specific incidence rates for males were 5.5, 15.0 and 15.3 in 2001-2014 for the three age groups. The corresponding rates for females were 1.2, 3.1 and 5.5.
 
The application of CT increased over time for all age groups. The proportions of CT for the three age groups were 56, 51 and 34% in 1989-2000, as compared with 81, 73 and 53% in 2001-2014. The corresponding proportions for IT were 21, 13 and 17% in 1989-2000, as compared with 2, 1 and 4% in 2001-2014. Lastly, the corresponding proportions for pts who did not receive therapy were 23, 36 and 49% in 1989-2000, as compared with 17, 26 and 42% in 2001-2014.
 
Overall, when corrected for age and sex, pts diagnosed in 2001-2014 had 49% lower excess mortality during the first 10 years after HCL diagnosis, as compared with pts diagnosed in 1989-2000 (P=.005). Ten-year RS (95% confidence intervals) was impressive for pts age 18-59, namely 92% (88% - 96%) and 98% (94% - 100%; P=.176) in the first and last period, respectively (Fig 1a). Most of the significant improvement was observed in pts age ≥60. More specifically, 10-year RS for pts age 60-69 increased from 82% (71% - 92%) to 99% (89% - 106%; P=.009; Fig 1b), and for pts age ≥70 from 67% (49% - 86%) to 85% (68% - 102%; P=.366; Fig 1c) between the first and last periods. In addition, older age (P<.001), but not sex (P=.058), was associated with higher excess mortality.

Conclusion
The incidence of HCL remained stable during a 26-year period in the Netherlands. RS for pts diagnosed in the period 2001-2014 eventually reached a plateau, indicating that by then their survival is comparable to that of the general population. Survival was already excellent for younger patients throughout the entire study period. Survival improvement was most pronounced for pts age ≥60, although it was not statistically significant for pts age ≥70. This could be explained by the increased use CT over time. Population-based cancer registries are useful instruments to assess outcomes of pts rarely included in clinical trials.

Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical

Keyword(s): epidemiology, Treatment, Survival, Hairy cell leukemia

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