scholarly journals Plasma Cell Leukemia: A Retrospective Review of Cases at Monter Cancer Center/Northwell Health Cancer Institute 2014-2019

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 19-19
Author(s):  
Christina Cotte ◽  
Monique Hartley-Brown
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Working Group ◽  
Drug Evaluation ◽  
Plasma Cell Leukemia ◽  
Cancer Center ◽  
Cell Leukemia ◽  
Treatment Regimens ◽  
The Impact ◽  
International Myeloma Working Group

Title: Plasma Cell Leukemia: A retrospective review of cases at Monter Cancer Center/Northwell Health Cancer Institute 2014-2019 Background: Plasma cell leukemia (PCL) is an aggressive form of multiple myeloma with an acute onset and a median overall survival (OS) of 13 months.1,2 The defining criteria for PCL is in flux. Prior criteria supported diagnosis of PCL based on the presence of at least 20% clonal plasma cells in the peripheral blood3, while more recent data suggest the use of 5% based on cases with 5-19%.1 The benefit of novel targeted therapy in patients with PCL has begun to be elucidated.2,4 It is difficult to perform prospective studies on patients with PCL due to its fulminant course1,2, changing perspectives on diagnostic criteria1,2,3, and the fact that there is no clear guidance as to what treatment regimens work best in these patients. With the emergence of novel treatment options, such as Daratumumab, the impact on survival in these patients is important to evaluate.2 Aim: Our goal is to gather more information about the treatment approach to patients with PCL in an effort to identify regimens associated with better OS. We hypothesize that patients diagnosed with PCL have improved outcomes when treated with newer FDA approved therapies2, such as Daratumumab2,5,6, compared with traditional therapy. Methods: Using a retrospective study approach, we performed a chart review of patients diagnosed with PCL (primary and secondary) in the Northwell Health system from 2014-2019. Patients were identified at a single center site - Monter Cancer Center. Using the electronic medical records (EMR), patient chart analysis was done to collect baseline patient demographics, diagnoses, cytogenetics, fluorescence in-situ hybridization (FISH) results, laboratory results, treatment regimens, time to progression (TTP), progression-free survival (PFS), and OS. All included patients have a diagnosis of PCL, defined as per the Internal Myeloma Working Group (IMWG) diagnostic criteria.7,8,9 We will correlate survival outcomes (PFS and OS) to treatment regimen used. Results: A total of 10 patients with PCL has been identified. Fifty percent of the patients were male. Ages ranged from 47 - 82 years with a mean age of 65.2 and a median age of 62. Further analysis is in progress. Conclusion: PCL is an aggressive malignancy and an area of unmet need.2,4,7 Due to its fulminant course and the lack of prospective clinical trials in PCL, the optimal therapeutic approach remains controversial.2 This study may potentially highlight the impact of newer therapies for PCL. We propose that further multi-institutional prospective studies in PCL will be important to determine better treatment. References Ravi P, Kumar SK. (2018). Revised diagnostic criteria for plasma cell leukemia: results of Mayo Clinic study with comparison of outcomes to multiple myeloma. Blood Cancer, 8(12). Ngu S, et al. (2019). Primary plasma cell leukemia: A case report and review of the literature. Clin Case Rep, 7(9). Kyle RA, et al. (1974). Plasma cell leukemia. Report on 17 cases. Arch Intern Med, 133(5). Gonsalves WI, et al. (2018). Combination therapy incorporating Bcl-2 inhibition with Venetoclax for the treatment of refractory primary plasma cell leukemia with t (11;14). Eur J Haemotol, 100(2). Center for Drug Evaluation and Research. (2019, September 26). FDA approves daratumumab for transplant-eligible multiple myeloma. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-daratumumab-transplant-eligible-multiple-myeloma. Center for Drug Evaluation and Research. (2020, May 1). FDA approves daratumumab and hyaluronidase-fihj for multiple myeloma. https://www.fda.gov/drugs/drug-approvals-and-databases/fda-approves-daratumumab-and-hyaluronidase-fihj-multiple-myeloma. De Larrea CF, Kyle RA, et al. on behalf of the International Myeloma Working Group (2013). Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria, and treatment recommendations by the International Myeloma Working Group (IMWG). Leukemia 27. Kumar SK, et al. (2016). International Myeloma Working Group consensus criteria for response and minimal residual disease assessment in multiple myeloma. Lancet Oncol. 17(8), e328-e346. Gundesen MT, et al. (2019). Plasma Cell Leukemia: Definition, Presentation, and Treatment. Cur Oncol Rep. 21(1). Disclosures No relevant conflicts of interest to declare.

scholarly journals Proteome alterations associated with transformation of multiple myeloma to secondary plasma cell leukemia

Oncotarget ◽  
2016 ◽  
Vol 8 (12) ◽  
pp. 19427-19442 ◽  
Author(s):  
Alexey Zatula ◽  
Aida Dikic ◽  
Celine Mulder ◽  
Animesh Sharma ◽  
Cathrine B. Vågbø ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Secondary Plasma

scholarly journals Pixantrone demonstrates significant in vitro activity against multiple myeloma and plasma cell leukemia

2019 ◽  
Vol 98 (11) ◽  
pp. 2569-2578
Author(s):  
Ella Willenbacher ◽  
Karin Jöhrer ◽  
Wolfgang Willenbacher ◽  
Brigitte Flögel ◽  
Richard Greil ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Cell Lines ◽  
Plasma Cell ◽  
Myeloma Cell ◽  
Plasma Cell Leukemia ◽  
Phase Iii ◽  
In Vivo Model ◽  
Cell Leukemia ◽  
Cam Assay

Abstract Treatment results for multiple myeloma and plasma cell leukemia have considerably improved, but cure remains elusive and establishing new therapeutic approaches constitutes a major unmet clinical need. We analyzed the anti-myeloma properties of the aza-anthracenedione pixantrone which has been successfully used in a phase III study for the treatment of patients with aggressive non-Hodgkin’s lymphoma as monotherapy as well as in combination regimes in vitro and in an adapted in vivo model (ex ovo chicken chorioallantoic membrane (CAM) assay). Pixantrone significantly inhibited proliferation and metabolic activity of all investigated myeloma cell lines. Importantly, anti-myeloma effects were more pronounced in tumor cell lines than in stromal cells, mesenchymal stem cells, and peripheral blood mononuclear cells of healthy controls. Apoptosis of myeloma cell lines was observed only after a 7-day incubation period, indicating a fast cytostatic and a slower cytotoxic effect of this drug. Pixantrone reduced the viability of primary plasma cells of patients and induced downregulation of myeloma-cell growth in the CAM assay. Additionally, we demonstrate in vitro synergism between pixantrone and the histone deacetylase inhibitor panobinostat with respect to its anti-proliferative features. From these data, we conclude that systematic investigations of the clinical usefulness of pixantrone in the framework of controlled clinical trials are clearly indicated (e.g., in penta-refractory patients).

Lack ofBCL10 mutations in multiple myeloma and plasma cell leukemia

2001 ◽  
Vol 30 (4) ◽  
pp. 402-406 ◽  
Author(s):  
Lee-Yung Shih ◽  
Jen-Fen Fu ◽  
Sheila A. Shurtleff ◽  
Stephan W. Morris ◽  
James R. Downing
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia

The sitotoxic effect of gemcitabine on multiple myeloma (RPMI-8226) and Ig G plasma cell leukemia (ARH 77) cell lines

2012 ◽  
Vol 54 (4) ◽  
pp. 263 ◽  
Author(s):  
Zehra Coban ◽  
Ferit Avcu ◽  
Ali Ural ◽  
Okan Kuzhan ◽  
Sefik Guran
Keyword(s):  
Multiple Myeloma ◽  
Cell Lines ◽  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Rpmi 8226 ◽  
Ig G

Cytogenetic and fluorescence in situ hybridization studies in 60 patients with multiple myeloma and plasma cell leukemia

2004 ◽  
Vol 148 (1) ◽  
pp. 71-76 ◽  
Author(s):  
Elisabet Lloveras ◽  
Isabel Granada ◽  
Lurdes Zamora ◽  
Blanca Espinet ◽  
Lourdes Florensa ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
In Situ Hybridization ◽  
Fluorescence In Situ Hybridization ◽  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia

A Pilot Study of Lenalidomide and Dexamethasone as First Line Therapy in Patients with Primary Plasma Cell Leukemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4951-4951
Author(s):  
Pellegrino Musto ◽  
Maria Teresa Petrucci ◽  
Fortunato Morabito ◽  
Francesco Nobile ◽  
Fiorella D'Auria ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Early Response ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Primary Plasma Cell Leukemia

Abstract Abstract 4951 Background Primary Plasma Cell Leukemia (PPCL) is an aggressive, rare variant of multiple myeloma, with clinical, molecular and phenotypic peculiarities, which accounts approximately for 2% to 4% of all myeloma diagnoses. The prognosis of PPCL patients is usually poor, with less than half of patients responding to conventional chemotherapy and a median survival of 7 months. Even by using autologous or allogeneic transplant procedures, survival generally does not exceed three years. Bortezomib has recently provided some promising results in this setting, but, given all the above, new treatments for PPCL are greatly awaited. Lenalidomide is a new immunomodulating agent with great efficacy in multiple myeloma, especially when associated with dexamethasone or other drugs. There are, indeed, some sporadic case reports of PPCL patients treated with lenalidomide as salvage therapy, but no data are currently available on the use of this drug as first line therapy in this disease. Patients and Methods On March, 2009, we started an open label, prospective, multicenter, exploratory, single arm, two-stage study aiming to evaluate safety and antitumor activity of the lenalidomide/low dose dexamethasone combination (Rd), as first line therapy in patients with PPCL. The primary endpoint was early response rate according to International Uniform Criteria. The secondary endpoints were TTP, PFS, OS, percentage of eligible PPCL patients able to collect peripheral blood stem cells and to undergo autologous or allogeneic stem cells transplantation after Rd, serious and severe adverse event rate. According to this study protocol, all eligible, newly diagnosed adult patients with PPCL receive Lenalidomide at a dose of 25 mg daily for 21 days every 28 days. Oral dexamethasone is administered at a dose of 40 mg daily on days 1, 8, 15, and 22 for each 28-day cycle. After 4 cycles, patients who achieve at least PR and not eligible for autologous or allogeneic stem cell transplantation, continue with Rd until clinically appropriate (disease progression, unacceptable toxicity, patient's decision to leave the protocol). In these patients, a maintenance dose of lenalidomide alone equal to 10 mg/die days 1-21 every month is considered after at least 8 full dose Rd cycles. Patients responding after 4 Rd cycles and eligible for transplant procedures, proceed according to single Centre transplant policy. Patients not responding after 4 cycles or progressing under Rd treatment are considered off-study. Appropriate contraception methods and anti-thrombotic prophylaxis are planned. Results Four enrolled patients (1 male, 3 female, mean age 65 years, range 58-69) are currently evaluable for early response. All had unfavourable cytogenetics, including del13, t(4;14), t (14;16), or a complex karyotype. Circulating plasma cells ranged from 4.4 to 9.2 ×10e9/l. One patient had at baseline a moderate degree of renal failure (serum creatinine levels 2 mg/dl). After at least 2 Rd cycles (range 2-4), two PR and two VGPR were achieved (overall response rate 100%), with disappearance or near complete reduction of circulating plasma cells in all cases. The most relevant toxicities were grade 3 neutropenia and pneumonia, occurring in one patient and resolved by appropriate lenalidomide dose reduction, introduction of G-CSF and antibiotic therapy. One patient died in PR, due to causes unrelated to PPCL or treatment. As, according to the Simon, two-stage design adopted, more than two responses occurred within the first ten patients enrolled (stage 1), a total of 22 PPCL subjects will be accrued to complete the stage 2 of the trial. Conclusions These findings, though very preliminary, suggest that the combination of lenalidomide and dexamethasone may be a safe and promising initial therapy for PPCL patients, which can rapidly control the disease and could permit to perform following single patient-adapted therapeutic strategies. An update of this study, including molecular data, a larger number of patients and a longer follow-up, will be presented at the Meeting. Disclosures Musto: Janssen-Cilag: Honoraria; Celgene: Honoraria, Research Funding. Off Label Use: Lenalidomide is approved in Italy for advanced multiple myeloma, not for plasma cell leukemia. This is a clinical trial registered at AIFA (Italian regulatory Agency for Drugs), EudraCT No. 2008-003246 28. Petrucci:Janssen-Cilag: Honoraria; Celgene: Honoraria. Morabito:Celgene: Honoraria; Janssen-Cilag: Honoraria. Cavo:Celgene: Honoraria; Janssen-Cilag: Honoraria. Boccadoro:Celgene: Honoraria; Janssen-Cilag: Honoraria. Palumbo:Celgene: Honoraria; Janssen-Cilag: Honoraria.

Real World Data In Myeloma: Experiences From The Swedish Population-Based Registry On 2494 Myeloma Patients Diagnosed 2008-2011

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1972-1972 ◽  
Author(s):  
Cecilie Blimark ◽  
Erik Holmberg ◽  
Gunnar Juliusson ◽  
Hareth Nahi ◽  
Forsberg Karin ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Real World ◽  
Clinical Intervention ◽  
Population Based ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
First Report ◽  
One Year ◽  
The Impact

Abstract Introduction The Swedish Myeloma Registry (SMR) is a prospective observational registry designed to document real-world management and outcomes in newly diagnosed myeloma, with the purpose to improve the quality of the management of patients in Sweden. Population-based registries may provide complementary information on the management of patients to that of clinical intervention trials. With high representation and excellent data quality we can present valuable information in a whole population and reduce the impact of selection on outcome and reduce the subsequent problem with extrapolating data from clinical intervention studies on non-study populations. Methods The registry comprises web-reported data on all patients diagnosed with myeloma, plasmocytoma, and plasma cell leukemia from 2008 in Sweden, at time of diagnosis and after one year of follow-up. Coverage is analyzed through the compulsory Swedish Cancer Registry. Survival is achieved from the Swedish Tax Agency. Missing data are actively requested. This first report contains data on patients diagnosed between 2008 and 2011 with follow-up after one year on patients with symptomatic disease 2008-2010, with a follow-up through the end of 2012. Analyses of incidence, patient characteristics at baseline, proportion of patients given intensive treatment, obtaining very good partial remission (VGPR) and overall survival (OS) were estimated. Results Clinical data at baseline was available for 2494 patients (96% coverage)and 1- year follow-up data on 1427 patients (90% of all symptomatic cases initially reported), from 70 different centers in Sweden. The age adjusted incidence was 6.5 myeloma cases per 100 000 inhabitants and year. The median age was 70 years for men, and 73 years for women (34% younger than 66 years). At diagnosis, 76% were reported as symptomatic myeloma, 18% as smouldering myeloma, 5% plasmocytoma and 1% plasma cell leukemia. IgG-myeloma was most common (59%), followed by IgA (21%), Bence-Jones (13%), non-secretory (4%), IgD and IgM both less than 1%. Among symptomatic myeloma (n=1910), 76% had osteolytic lesions or compression fractures at diagnosis. Anemia (defined as hemoglobin levels below 10 g/dl) was seen in 33%, impaired kidney function (s-creatinin levels above 173 mmol/l) in 18%, and hypercalcemia in 21% at the time of diagnosis. In patients were ISS was available, 23%, 45% and 32% were in stage I, II, and III, respectively. Previous MGUS was known in 13 % of patients. Overall, 81 % of patients 65 years or younger received autologous stem cell transplantation (ASCT) and 4% of the elderly population. In the patients aged 65 years and younger, 63% of patients received one of the newer drugs in the first year of treatment, for the patients 66 to 80 years the number was 56%, and 25% of patients above 80 years. Throughout the study period, an increase in VGPR-rate on initial treatment was observed, more pronounced in younger patients (<66 years), from 35% in 2008 to 46% in 2010. For patients >65 years, the VGPR-rate increased from 17 to 27%. After a median of follow-up time of three years, OS was 63%. There was a significant difference in absolute and relative survival between younger and older patients. In symptomatic myeloma, patients 65 years or younger had an expected 3-year survival of 76% and in patients 66 years and above it was 50% (Figure). The relative 3-year survival for patients with asymptomatic patients was 81%. Discussion SMR is an instrument for increased quality in the management of plasma cell neoplasms in Sweden. This first report from the registry shows very high coverage and good adherence to guidelines in all regions of Sweden, both in diagnostics and treatment. A great effort is made to make the SMR complete and to present population-based data on management and outcome in Sweden. Longer follow-up is needed to address the question of the impact of new treatment options on the survival. The registry gives a great opportunity to perform population-based research of high quality based on the acceptance of the registry among treating physicians. Disclosures: Turesson: Celgene Corp: Honoraria.

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