PLASMA CELL LEUKEMIA: A 10-YEAR SINGLE CENTER EXPERIENCE
(Abstract release date: 05/19/16)
EHA Library. Pinto A. 06/09/16; 134894; PB1994
Ms. Ana Luisa Pinto
Contributions
Contributions
Abstract
Abstract: PB1994
Type: Publication Only
Background
Plasma cell leukemia (PCL) is a rare disorder representing less than 5% of malignant plasma cell diseases and defined by the presence of clonal plasma cells in the peripheral blood with an absolute count of >2x109/L and/or >20% of the white blood cells. It presents either as primary disease (pPCL) or as a secondary leukemic transformation (sPCL) of multiple myeloma, and usually has an aggressive course and poor outcome.
Aims
To evaluate clinical features, prognostic factors and treatment outcome in patients with PCL.
Methods
A single center retrospective case-series study was performed. Data from patients diagnosed with PCL between January 2006 and December 2015 were collected and analysed.
Results
We identified 15 patients with PCL (5 male/10 female, median age 68 years, range 44-78) of whom 11 had pPCL and 4 sPCL. In sPCL the median time to leukemic progression was 22.3 months, with a median of 3 previous therapeutic lines.The subtype of monoclonal component found at diagnosis was IgG (n=7), IgA (n=2), IgE (n=1), light chain only (n=3) and non-secretory (n=2). Median plasma cell count in peripheral blood was 4.05x109/L (range 0.3-60.5) with a median proportion of plasma cells of 32% (range 11-85).At baseline, most of the patients had poor performance status with ECOG³2 (n=9), advanced stage disease classified at stage III according to International Staging System (n=11) and Durie-Salmon Staging System (n=10), and at least one end-organ damage (n=14). Extramedullary disease was present in 5 cases. Elevated lactate dehydrogenase was observed in 11 patients (median 338U/L, range 118-2610, N<248U/L) and β2-microglobulin in 12 (median 17.7mg/L, range 1.0-62.1). In 8 patients cytogenetic studies were carried out by conventional cytogenetic analysis and by FISH. Seven of them presented high risk cytogenetic alterations.Four patients received anthracycline-based regimens as first-line treatment, 2 single alkylating agents and 6 bortezomib or lenalidomide as additional or unique treatments. In 3 patients, with very aggressive disease and poor performance status, early death occurred, allowing only palliative treatment with steroids. Two patients underwent autologous hematopoietic stem cell transplantation after first line treatment. Eight patients achieved complete or partial response.The median overall survival (OS) was 4.5 months and OS at 2 years was 12.5%. Significantly longer OS was observed in patients responding to first-line treatment versus those who did not respond (median 9.6 vs 0.9 months, p=0.002); in patients who received bortezomib during the course of the disease (median 23.8 vs 0.9 months, p=0.022), and in patients with good performance status (ECOG <2) at the time of diagnosis (9.6 vs 4.1 months, p=0.024).
Conclusion
PCL is a rare disease with a poor prognosis, aggressive clinicobiological features and a low response rate to conventional treatment. Although our conclusions are limited by the small sample size, our study shows that good performance status and response to first-line treatment present a positive impact on survival. The use of bortezomib also appears to improve outcomes by lengthening OS.
Session topic: E-poster
Keyword(s): Bortezomib, Leukemia, Plasma cells, Prognostic factor
Type: Publication Only
Background
Plasma cell leukemia (PCL) is a rare disorder representing less than 5% of malignant plasma cell diseases and defined by the presence of clonal plasma cells in the peripheral blood with an absolute count of >2x109/L and/or >20% of the white blood cells. It presents either as primary disease (pPCL) or as a secondary leukemic transformation (sPCL) of multiple myeloma, and usually has an aggressive course and poor outcome.
Aims
To evaluate clinical features, prognostic factors and treatment outcome in patients with PCL.
Methods
A single center retrospective case-series study was performed. Data from patients diagnosed with PCL between January 2006 and December 2015 were collected and analysed.
Results
We identified 15 patients with PCL (5 male/10 female, median age 68 years, range 44-78) of whom 11 had pPCL and 4 sPCL. In sPCL the median time to leukemic progression was 22.3 months, with a median of 3 previous therapeutic lines.The subtype of monoclonal component found at diagnosis was IgG (n=7), IgA (n=2), IgE (n=1), light chain only (n=3) and non-secretory (n=2). Median plasma cell count in peripheral blood was 4.05x109/L (range 0.3-60.5) with a median proportion of plasma cells of 32% (range 11-85).At baseline, most of the patients had poor performance status with ECOG³2 (n=9), advanced stage disease classified at stage III according to International Staging System (n=11) and Durie-Salmon Staging System (n=10), and at least one end-organ damage (n=14). Extramedullary disease was present in 5 cases. Elevated lactate dehydrogenase was observed in 11 patients (median 338U/L, range 118-2610, N<248U/L) and β2-microglobulin in 12 (median 17.7mg/L, range 1.0-62.1). In 8 patients cytogenetic studies were carried out by conventional cytogenetic analysis and by FISH. Seven of them presented high risk cytogenetic alterations.Four patients received anthracycline-based regimens as first-line treatment, 2 single alkylating agents and 6 bortezomib or lenalidomide as additional or unique treatments. In 3 patients, with very aggressive disease and poor performance status, early death occurred, allowing only palliative treatment with steroids. Two patients underwent autologous hematopoietic stem cell transplantation after first line treatment. Eight patients achieved complete or partial response.The median overall survival (OS) was 4.5 months and OS at 2 years was 12.5%. Significantly longer OS was observed in patients responding to first-line treatment versus those who did not respond (median 9.6 vs 0.9 months, p=0.002); in patients who received bortezomib during the course of the disease (median 23.8 vs 0.9 months, p=0.022), and in patients with good performance status (ECOG <2) at the time of diagnosis (9.6 vs 4.1 months, p=0.024).
Conclusion
PCL is a rare disease with a poor prognosis, aggressive clinicobiological features and a low response rate to conventional treatment. Although our conclusions are limited by the small sample size, our study shows that good performance status and response to first-line treatment present a positive impact on survival. The use of bortezomib also appears to improve outcomes by lengthening OS.
Session topic: E-poster
Keyword(s): Bortezomib, Leukemia, Plasma cells, Prognostic factor
Abstract: PB1994
Type: Publication Only
Background
Plasma cell leukemia (PCL) is a rare disorder representing less than 5% of malignant plasma cell diseases and defined by the presence of clonal plasma cells in the peripheral blood with an absolute count of >2x109/L and/or >20% of the white blood cells. It presents either as primary disease (pPCL) or as a secondary leukemic transformation (sPCL) of multiple myeloma, and usually has an aggressive course and poor outcome.
Aims
To evaluate clinical features, prognostic factors and treatment outcome in patients with PCL.
Methods
A single center retrospective case-series study was performed. Data from patients diagnosed with PCL between January 2006 and December 2015 were collected and analysed.
Results
We identified 15 patients with PCL (5 male/10 female, median age 68 years, range 44-78) of whom 11 had pPCL and 4 sPCL. In sPCL the median time to leukemic progression was 22.3 months, with a median of 3 previous therapeutic lines.The subtype of monoclonal component found at diagnosis was IgG (n=7), IgA (n=2), IgE (n=1), light chain only (n=3) and non-secretory (n=2). Median plasma cell count in peripheral blood was 4.05x109/L (range 0.3-60.5) with a median proportion of plasma cells of 32% (range 11-85).At baseline, most of the patients had poor performance status with ECOG³2 (n=9), advanced stage disease classified at stage III according to International Staging System (n=11) and Durie-Salmon Staging System (n=10), and at least one end-organ damage (n=14). Extramedullary disease was present in 5 cases. Elevated lactate dehydrogenase was observed in 11 patients (median 338U/L, range 118-2610, N<248U/L) and β2-microglobulin in 12 (median 17.7mg/L, range 1.0-62.1). In 8 patients cytogenetic studies were carried out by conventional cytogenetic analysis and by FISH. Seven of them presented high risk cytogenetic alterations.Four patients received anthracycline-based regimens as first-line treatment, 2 single alkylating agents and 6 bortezomib or lenalidomide as additional or unique treatments. In 3 patients, with very aggressive disease and poor performance status, early death occurred, allowing only palliative treatment with steroids. Two patients underwent autologous hematopoietic stem cell transplantation after first line treatment. Eight patients achieved complete or partial response.The median overall survival (OS) was 4.5 months and OS at 2 years was 12.5%. Significantly longer OS was observed in patients responding to first-line treatment versus those who did not respond (median 9.6 vs 0.9 months, p=0.002); in patients who received bortezomib during the course of the disease (median 23.8 vs 0.9 months, p=0.022), and in patients with good performance status (ECOG <2) at the time of diagnosis (9.6 vs 4.1 months, p=0.024).
Conclusion
PCL is a rare disease with a poor prognosis, aggressive clinicobiological features and a low response rate to conventional treatment. Although our conclusions are limited by the small sample size, our study shows that good performance status and response to first-line treatment present a positive impact on survival. The use of bortezomib also appears to improve outcomes by lengthening OS.
Session topic: E-poster
Keyword(s): Bortezomib, Leukemia, Plasma cells, Prognostic factor
Type: Publication Only
Background
Plasma cell leukemia (PCL) is a rare disorder representing less than 5% of malignant plasma cell diseases and defined by the presence of clonal plasma cells in the peripheral blood with an absolute count of >2x109/L and/or >20% of the white blood cells. It presents either as primary disease (pPCL) or as a secondary leukemic transformation (sPCL) of multiple myeloma, and usually has an aggressive course and poor outcome.
Aims
To evaluate clinical features, prognostic factors and treatment outcome in patients with PCL.
Methods
A single center retrospective case-series study was performed. Data from patients diagnosed with PCL between January 2006 and December 2015 were collected and analysed.
Results
We identified 15 patients with PCL (5 male/10 female, median age 68 years, range 44-78) of whom 11 had pPCL and 4 sPCL. In sPCL the median time to leukemic progression was 22.3 months, with a median of 3 previous therapeutic lines.The subtype of monoclonal component found at diagnosis was IgG (n=7), IgA (n=2), IgE (n=1), light chain only (n=3) and non-secretory (n=2). Median plasma cell count in peripheral blood was 4.05x109/L (range 0.3-60.5) with a median proportion of plasma cells of 32% (range 11-85).At baseline, most of the patients had poor performance status with ECOG³2 (n=9), advanced stage disease classified at stage III according to International Staging System (n=11) and Durie-Salmon Staging System (n=10), and at least one end-organ damage (n=14). Extramedullary disease was present in 5 cases. Elevated lactate dehydrogenase was observed in 11 patients (median 338U/L, range 118-2610, N<248U/L) and β2-microglobulin in 12 (median 17.7mg/L, range 1.0-62.1). In 8 patients cytogenetic studies were carried out by conventional cytogenetic analysis and by FISH. Seven of them presented high risk cytogenetic alterations.Four patients received anthracycline-based regimens as first-line treatment, 2 single alkylating agents and 6 bortezomib or lenalidomide as additional or unique treatments. In 3 patients, with very aggressive disease and poor performance status, early death occurred, allowing only palliative treatment with steroids. Two patients underwent autologous hematopoietic stem cell transplantation after first line treatment. Eight patients achieved complete or partial response.The median overall survival (OS) was 4.5 months and OS at 2 years was 12.5%. Significantly longer OS was observed in patients responding to first-line treatment versus those who did not respond (median 9.6 vs 0.9 months, p=0.002); in patients who received bortezomib during the course of the disease (median 23.8 vs 0.9 months, p=0.022), and in patients with good performance status (ECOG <2) at the time of diagnosis (9.6 vs 4.1 months, p=0.024).
Conclusion
PCL is a rare disease with a poor prognosis, aggressive clinicobiological features and a low response rate to conventional treatment. Although our conclusions are limited by the small sample size, our study shows that good performance status and response to first-line treatment present a positive impact on survival. The use of bortezomib also appears to improve outcomes by lengthening OS.
Session topic: E-poster
Keyword(s): Bortezomib, Leukemia, Plasma cells, Prognostic factor
{{ help_message }}
{{filter}}