A RETROSPECTIVE ANALYSIS OF SPLENIC MARGINAL ZONE LYMPHOMA: PROGNOSTIC FACTORS, ROLE OF WATCH AND WAIT, AND THERAPEUTIC APPROACHES IN THE RITUXIMAB ERA
(Abstract release date: 05/21/15)
EHA Library. Perrone S. 06/12/15; 102952; PB1785
Disclosure(s): Sapienza UniversityHematology, Department of Cellular Biotechnologies and Hematology.
Salvatore Perrone
Contributions
Contributions
Abstract
Abstract: PB1785
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoma recognized as a distinct entity in the WHO classification. Arcaini’s scoring system (Blood, 2006) - based on Hgb <12 g/dL, albumin <3.5 g/dL, elevated LDH - is useful to stratify patients prognostically. Given the lack of standard criteria guiding treatment initiation, the watch and wait (W&W) approach in asymptomatic patients is recommended. In symptomatic patients it has been suggested that, apart from splenectomy, rituximab +/- chemotherapy is the best option.
Aims
The aims of our study were to identify risk factors at diagnosis, to assess the progression rate within a W&W strategy, and to analyze the outcome of different therapies in the post-rituximab era.
Methods
Results
The median age at diagnosis was 66 years. The male/female ratio was 1.1. HCV antibodies were positive in 3.7% of cases. The 10-year overall survival was 93% (CI: 84.7-100%). Notably, no patient died of disease progression. The 5- and 10-year progression-free survival (PFS) were 77% and 62%, respectively. In univariate analysis, negative predictors of a worse PFS were splenomegaly (>18cm) and bone marrow infiltration (>30%). Patients with a low Arcaini score had a better 5-year PFS (87%) than those with an intermediate (76%) or high (44%) risk score (p value=0.01). Fifty asymptomatic patients underwent a W&W program. The median PFS of this population was 45 months; at 10 years, 17% of patients are still on W&W (FIG 1). Sixty-five patients, either at diagnosis or after a W&W period, were treated; those treated with splenectomy or R-chemo first line had similar results, while those treated only with chemo had an inferior outcome. However, when analyzing separately patients with a score <1, splenectomy alone resulted in a highly significant PFS advantage compared to the other treatment approaches (FIG 2).
Summary
This real-life single center study offers an insight into the natural history of indolent SMZL, confirming its very good prognosis. We found a negative prognostic impact of a marked splenomegaly and marrow infiltration. At variance from previous reports, we did not observe a high prevalence of HCV, especially as this is a Center/South-Italian case series. Moreover, we confirm that Arcaini’s staging system, that defines 3 separate risk groups, is a powerful prognostic stratificator. The W&W approach allows a median PFS of 45 months, longer than that reported in follicular lymphoma patients. Finally, our data confirm the inferiority of chemotherapy alone against splenectomy and R-chemo. The subgroup of low risk patients treated only with splenectomy fared very well. For such patients, splenectomy could remain the best first-line approach even in the rituximab era. Prospective studies are needed to confirm these results.
Keyword(s): Clinical data, Hepatitis C virus, Rituximab, Splenic marginal zone lymphoma
Session topic: Publication Only
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoma recognized as a distinct entity in the WHO classification. Arcaini’s scoring system (Blood, 2006) - based on Hgb <12 g/dL, albumin <3.5 g/dL, elevated LDH - is useful to stratify patients prognostically. Given the lack of standard criteria guiding treatment initiation, the watch and wait (W&W) approach in asymptomatic patients is recommended. In symptomatic patients it has been suggested that, apart from splenectomy, rituximab +/- chemotherapy is the best option.
Aims
The aims of our study were to identify risk factors at diagnosis, to assess the progression rate within a W&W strategy, and to analyze the outcome of different therapies in the post-rituximab era.
Methods
: We retrospectively examined the clinical files of 83 patients with SMZL managed at our center in Rome between 2000 and 2013. Patients were stratified according to Arcaini’s scoring system. Asymptomatic patients were managed with a W&W policy. Splenectomy was performed in 21 patients with a symptomatic spleen enlargement and limited bone marrow or nodal involvement. Patients not eligible for a splenectomy or with a more generalized disease were treated with chemotherapy alone (12) and, after its introduction, with rituximab + chemotherapy (R-chemo) (8).
Results
The median age at diagnosis was 66 years. The male/female ratio was 1.1. HCV antibodies were positive in 3.7% of cases. The 10-year overall survival was 93% (CI: 84.7-100%). Notably, no patient died of disease progression. The 5- and 10-year progression-free survival (PFS) were 77% and 62%, respectively. In univariate analysis, negative predictors of a worse PFS were splenomegaly (>18cm) and bone marrow infiltration (>30%). Patients with a low Arcaini score had a better 5-year PFS (87%) than those with an intermediate (76%) or high (44%) risk score (p value=0.01). Fifty asymptomatic patients underwent a W&W program. The median PFS of this population was 45 months; at 10 years, 17% of patients are still on W&W (FIG 1). Sixty-five patients, either at diagnosis or after a W&W period, were treated; those treated with splenectomy or R-chemo first line had similar results, while those treated only with chemo had an inferior outcome. However, when analyzing separately patients with a score <1, splenectomy alone resulted in a highly significant PFS advantage compared to the other treatment approaches (FIG 2).
Summary
This real-life single center study offers an insight into the natural history of indolent SMZL, confirming its very good prognosis. We found a negative prognostic impact of a marked splenomegaly and marrow infiltration. At variance from previous reports, we did not observe a high prevalence of HCV, especially as this is a Center/South-Italian case series. Moreover, we confirm that Arcaini’s staging system, that defines 3 separate risk groups, is a powerful prognostic stratificator. The W&W approach allows a median PFS of 45 months, longer than that reported in follicular lymphoma patients. Finally, our data confirm the inferiority of chemotherapy alone against splenectomy and R-chemo. The subgroup of low risk patients treated only with splenectomy fared very well. For such patients, splenectomy could remain the best first-line approach even in the rituximab era. Prospective studies are needed to confirm these results.
Keyword(s): Clinical data, Hepatitis C virus, Rituximab, Splenic marginal zone lymphoma
Session topic: Publication Only
Abstract: PB1785
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoma recognized as a distinct entity in the WHO classification. Arcaini’s scoring system (Blood, 2006) - based on Hgb <12 g/dL, albumin <3.5 g/dL, elevated LDH - is useful to stratify patients prognostically. Given the lack of standard criteria guiding treatment initiation, the watch and wait (W&W) approach in asymptomatic patients is recommended. In symptomatic patients it has been suggested that, apart from splenectomy, rituximab +/- chemotherapy is the best option.
Aims
The aims of our study were to identify risk factors at diagnosis, to assess the progression rate within a W&W strategy, and to analyze the outcome of different therapies in the post-rituximab era.
Methods
Results
The median age at diagnosis was 66 years. The male/female ratio was 1.1. HCV antibodies were positive in 3.7% of cases. The 10-year overall survival was 93% (CI: 84.7-100%). Notably, no patient died of disease progression. The 5- and 10-year progression-free survival (PFS) were 77% and 62%, respectively. In univariate analysis, negative predictors of a worse PFS were splenomegaly (>18cm) and bone marrow infiltration (>30%). Patients with a low Arcaini score had a better 5-year PFS (87%) than those with an intermediate (76%) or high (44%) risk score (p value=0.01). Fifty asymptomatic patients underwent a W&W program. The median PFS of this population was 45 months; at 10 years, 17% of patients are still on W&W (FIG 1). Sixty-five patients, either at diagnosis or after a W&W period, were treated; those treated with splenectomy or R-chemo first line had similar results, while those treated only with chemo had an inferior outcome. However, when analyzing separately patients with a score <1, splenectomy alone resulted in a highly significant PFS advantage compared to the other treatment approaches (FIG 2).
Summary
This real-life single center study offers an insight into the natural history of indolent SMZL, confirming its very good prognosis. We found a negative prognostic impact of a marked splenomegaly and marrow infiltration. At variance from previous reports, we did not observe a high prevalence of HCV, especially as this is a Center/South-Italian case series. Moreover, we confirm that Arcaini’s staging system, that defines 3 separate risk groups, is a powerful prognostic stratificator. The W&W approach allows a median PFS of 45 months, longer than that reported in follicular lymphoma patients. Finally, our data confirm the inferiority of chemotherapy alone against splenectomy and R-chemo. The subgroup of low risk patients treated only with splenectomy fared very well. For such patients, splenectomy could remain the best first-line approach even in the rituximab era. Prospective studies are needed to confirm these results.
Keyword(s): Clinical data, Hepatitis C virus, Rituximab, Splenic marginal zone lymphoma
Session topic: Publication Only
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoma recognized as a distinct entity in the WHO classification. Arcaini’s scoring system (Blood, 2006) - based on Hgb <12 g/dL, albumin <3.5 g/dL, elevated LDH - is useful to stratify patients prognostically. Given the lack of standard criteria guiding treatment initiation, the watch and wait (W&W) approach in asymptomatic patients is recommended. In symptomatic patients it has been suggested that, apart from splenectomy, rituximab +/- chemotherapy is the best option.
Aims
The aims of our study were to identify risk factors at diagnosis, to assess the progression rate within a W&W strategy, and to analyze the outcome of different therapies in the post-rituximab era.
Methods
: We retrospectively examined the clinical files of 83 patients with SMZL managed at our center in Rome between 2000 and 2013. Patients were stratified according to Arcaini’s scoring system. Asymptomatic patients were managed with a W&W policy. Splenectomy was performed in 21 patients with a symptomatic spleen enlargement and limited bone marrow or nodal involvement. Patients not eligible for a splenectomy or with a more generalized disease were treated with chemotherapy alone (12) and, after its introduction, with rituximab + chemotherapy (R-chemo) (8).
Results
The median age at diagnosis was 66 years. The male/female ratio was 1.1. HCV antibodies were positive in 3.7% of cases. The 10-year overall survival was 93% (CI: 84.7-100%). Notably, no patient died of disease progression. The 5- and 10-year progression-free survival (PFS) were 77% and 62%, respectively. In univariate analysis, negative predictors of a worse PFS were splenomegaly (>18cm) and bone marrow infiltration (>30%). Patients with a low Arcaini score had a better 5-year PFS (87%) than those with an intermediate (76%) or high (44%) risk score (p value=0.01). Fifty asymptomatic patients underwent a W&W program. The median PFS of this population was 45 months; at 10 years, 17% of patients are still on W&W (FIG 1). Sixty-five patients, either at diagnosis or after a W&W period, were treated; those treated with splenectomy or R-chemo first line had similar results, while those treated only with chemo had an inferior outcome. However, when analyzing separately patients with a score <1, splenectomy alone resulted in a highly significant PFS advantage compared to the other treatment approaches (FIG 2).
Summary
This real-life single center study offers an insight into the natural history of indolent SMZL, confirming its very good prognosis. We found a negative prognostic impact of a marked splenomegaly and marrow infiltration. At variance from previous reports, we did not observe a high prevalence of HCV, especially as this is a Center/South-Italian case series. Moreover, we confirm that Arcaini’s staging system, that defines 3 separate risk groups, is a powerful prognostic stratificator. The W&W approach allows a median PFS of 45 months, longer than that reported in follicular lymphoma patients. Finally, our data confirm the inferiority of chemotherapy alone against splenectomy and R-chemo. The subgroup of low risk patients treated only with splenectomy fared very well. For such patients, splenectomy could remain the best first-line approach even in the rituximab era. Prospective studies are needed to confirm these results.
Keyword(s): Clinical data, Hepatitis C virus, Rituximab, Splenic marginal zone lymphoma
Session topic: Publication Only
{{ help_message }}
{{filter}}