TREATMENT AND SURVIVAL OF PATIENTS WITH PRIMARY PLASMA CELL LEUKEMIA: A NATIONWIDE POPULATION-BASED STUDY AMONG 179 PATIENTS DIAGNOSED IN THE NETHERLANDS FROM 1989 TO 2015
Author(s): ,
Mirian Brink
Affiliations:
Research,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands
,
Otto Visser
Affiliations:
Registration,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands
,
Sonja Zweegman
Affiliations:
Hematology,VU University Medical Center,Amsterdam,Netherlands
,
Pieter Sonneveld
Affiliations:
Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
,
Annemiek Broijl
Affiliations:
Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
,
Niels van de Donk
Affiliations:
Hematology,VU University Medical Center,Amsterdam,Netherlands
Avinash Dinmohamed
Affiliations:
Research,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands;Public Health,Erasmus University Medical Center,Rotterdam,Netherlands;Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
EHA Library. Dinmohamed A. 06/16/18; 215601; PS1301
Avinash Dinmohamed
Avinash Dinmohamed
Contributions
Abstract

Abstract: PS1301

Type: Poster Presentation

Presentation during EHA23: On Saturday, June 16, 2018 from 17:30 - 19:00

Location: Poster area

Background

Primary plasma cell leukemia (pPCL) is a very rare plasma cell dyscrasia with a very poor outcome. At present, there is a paucity of data on the outcome of pPCL, as prospective intervention studies in pPCL are very scare. Population-based studies can provide valuable information complementary to prospective intervention studies to assess treatment strategies and outcome in patients at the population level.

Aims

The aim of this nationwide, population-based study was to assess trends in primary therapy and survival among pPCL patients diagnosed during a 27-year period in the Netherlands.

Methods

We selected all pPCL patients diagnosed between 1989-2015 from the nationwide Netherlands Cancer Registry. Patients were categorized into three periods (1989-2000, 2001-2007, and 2008-2015) and two age groups (≤65 v ≥66 years). The periods were chosen based on the implementation of autologous stem cell transplantation (ASCT) and novel agents for multiple myeloma (MM) in the Netherlands. Data on primary therapy—i.e. no therapy and anti-pPCL therapy with or without a SCT—were available for individual patients. The primary end point was overall survival (OS), defined as the date from pPCL diagnosis to all-cause death. Patients alive were censored at February 1, 2017. Multivariable evaluation of OS was performed using Cox regression. P<0.05 indicates statistical significance.

Results

Our analytical cohort included 179 pPCL patients (median age, 65 years; age range, 34-91 years; 53% males; 79% diagnosed in non-academic centers; 8% with a prior malignancy). Patient characteristics were comparable across the three study periods. Overall, the application of anti-PCL therapy (including SCT) increased modestly from 88 to 92% among patients aged ≤65 (P=0.640) and from 67 to 71% among patients aged ≥66 (P=0.047) between 1989-2000 and 2008-2015, respectively. As for no therapy, the corresponding proportions were 12 and 8% among patients aged ≤65 and 33 and 29% among patients aged ≥66, respectively. What was noteworthy was that the application of SCT increased from 23 to 59% among patients aged ≤65 (P=0.004) and from 0 to 12% among patients aged ≥66 (P=0.084) between 1989-2000 and 2008-2015, respectively. During follow-up, 155 (87%) patients died. The median OS increased from 12.2 to 28.4 months for patients aged ≤65 (P=0.012; Fig 1A) and from 2.0 to 5.3 months for patients aged ≥66 (P=0.044; Fig 1B) between 1989-2000 and 2008-2015, respectively. Overall, when adjusted for the covariates listed in Table 1, the multivariable Cox model demonstrated an improvement of survival over time and an adverse effect of older age. However, when information on treatment was added to that model, the wider use of therapy explained the improved survival over time.

Conclusion

In this nationwide, population-based study, we demonstrated that survival in pPCL improved over time. The improvement is largely explained by the wider application of SCT over time, especially among patients aged ≤65, and the introduction of novel agents. Collectively, the therapeutic advances achieved in MM seem to gradually translate into benefits for pPCL patients. Nevertheless, outcome remains disappointingly poor among patients aged ≥66. Therefore, prospective intervention studies in pPCL are imperative to further improve outcome.

Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical

Keyword(s): Myeloma, Population, Survival, Treatment

Abstract: PS1301

Type: Poster Presentation

Presentation during EHA23: On Saturday, June 16, 2018 from 17:30 - 19:00

Location: Poster area

Background

Primary plasma cell leukemia (pPCL) is a very rare plasma cell dyscrasia with a very poor outcome. At present, there is a paucity of data on the outcome of pPCL, as prospective intervention studies in pPCL are very scare. Population-based studies can provide valuable information complementary to prospective intervention studies to assess treatment strategies and outcome in patients at the population level.

Aims

The aim of this nationwide, population-based study was to assess trends in primary therapy and survival among pPCL patients diagnosed during a 27-year period in the Netherlands.

Methods

We selected all pPCL patients diagnosed between 1989-2015 from the nationwide Netherlands Cancer Registry. Patients were categorized into three periods (1989-2000, 2001-2007, and 2008-2015) and two age groups (≤65 v ≥66 years). The periods were chosen based on the implementation of autologous stem cell transplantation (ASCT) and novel agents for multiple myeloma (MM) in the Netherlands. Data on primary therapy—i.e. no therapy and anti-pPCL therapy with or without a SCT—were available for individual patients. The primary end point was overall survival (OS), defined as the date from pPCL diagnosis to all-cause death. Patients alive were censored at February 1, 2017. Multivariable evaluation of OS was performed using Cox regression. P<0.05 indicates statistical significance.

Results

Our analytical cohort included 179 pPCL patients (median age, 65 years; age range, 34-91 years; 53% males; 79% diagnosed in non-academic centers; 8% with a prior malignancy). Patient characteristics were comparable across the three study periods. Overall, the application of anti-PCL therapy (including SCT) increased modestly from 88 to 92% among patients aged ≤65 (P=0.640) and from 67 to 71% among patients aged ≥66 (P=0.047) between 1989-2000 and 2008-2015, respectively. As for no therapy, the corresponding proportions were 12 and 8% among patients aged ≤65 and 33 and 29% among patients aged ≥66, respectively. What was noteworthy was that the application of SCT increased from 23 to 59% among patients aged ≤65 (P=0.004) and from 0 to 12% among patients aged ≥66 (P=0.084) between 1989-2000 and 2008-2015, respectively. During follow-up, 155 (87%) patients died. The median OS increased from 12.2 to 28.4 months for patients aged ≤65 (P=0.012; Fig 1A) and from 2.0 to 5.3 months for patients aged ≥66 (P=0.044; Fig 1B) between 1989-2000 and 2008-2015, respectively. Overall, when adjusted for the covariates listed in Table 1, the multivariable Cox model demonstrated an improvement of survival over time and an adverse effect of older age. However, when information on treatment was added to that model, the wider use of therapy explained the improved survival over time.

Conclusion

In this nationwide, population-based study, we demonstrated that survival in pPCL improved over time. The improvement is largely explained by the wider application of SCT over time, especially among patients aged ≤65, and the introduction of novel agents. Collectively, the therapeutic advances achieved in MM seem to gradually translate into benefits for pPCL patients. Nevertheless, outcome remains disappointingly poor among patients aged ≥66. Therefore, prospective intervention studies in pPCL are imperative to further improve outcome.

Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical

Keyword(s): Myeloma, Population, Survival, Treatment

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