scholarly journals Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group

Leukemia ◽  
2012 ◽  
Vol 27 (4) ◽  
pp. 780-791 ◽  
Author(s):  
C Fernández de Larrea ◽  
◽  
R A Kyle ◽  
B G M Durie ◽  
H Ludwig ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Working Group ◽  
Consensus Statement ◽  
Treatment Recommendations ◽  
Plasma Cell Leukemia ◽  
Response Criteria ◽  
Cell Leukemia ◽  
International Myeloma Working Group

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 2021 European Myeloma Network review and consensus statement on smoldering multiple myeloma: how to distinguish (and manage) Dr. Jekyll and Mr. Hyde

Haematologica ◽  
2021 ◽  
Author(s):  
Pellegrino Musto ◽  
Monika Engelhardt ◽  
Jo Caers ◽  
Niccolo’ Bolli ◽  
Martin Kaiser ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Plasma Cell ◽  
Working Group ◽  
Consensus Statement ◽  
Smoldering Multiple Myeloma ◽  
Bone Marrow Plasma ◽  
Plasma Cell Disorder ◽  
Cell Disorder ◽  
International Myeloma Working Group

According to the updated International Myeloma Working Group criteria, smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder characterized by an M-component >3 g/dL, bone marrow plasma cell infiltration >10% and

scholarly journals Efficacy of Daratumumab (Dara)-Based Regimens for the Treatment of Plasma Cell Leukemia (PCL)

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 29-30
Author(s):  
Ricardo Parrondo ◽  
Muhamad Alhaj Moustafa ◽  
Craig B. Reeder ◽  
Taimur Sher ◽  
Vivek Roy ◽  
...  
Keyword(s):  
High Risk ◽  
Median Time ◽  
Plasma Cell ◽  
Research Funding ◽  
Consensus Statement ◽  
Single Agent ◽  
Plasma Cell Leukemia ◽  
Cell Transplant ◽  
Cell Leukemia ◽  
First Response

Background: PCL is a rare and aggressive form of multiple myeloma (MM). It is associated with high risk cytogenetics, aggressive disease biology and a dismal prognosis. Dara has revolutionized the treatment of MM leading to deep and durable responses as a single agent and in combination with other agents. We evaluated the efficacy of Dara-based regimens in the treatment of PCL. Methods: Clinical charts of primary and secondary PCL patients treated at the Mayo Clinic Cancer Center between 2012-2019 were reviewed. Survival was analyzed with the Kaplan-Meier method. Plasma cell leukemia was defined by ≥5% peripheral blood plasma cells and/or absolute plasma cell count ≥0.5 x109/L (based on IMWG consensus statement).1 Results: Thirty-one patients were identified (55% female) with a median age at PCL diagnosis of 66 y (range 38-83). Fifteen (48%) had primary (p) PCL, while 14 (52%) had secondary (s) PCL. Nineteen (61%) had high risk cytogenetics with 11 (45%) del 17p, 4 (13%) t(14;16), 14 (45%) 1q amplification/duplication and 3(10%) t(4;14). Twelve (39%) had t(11;14). Eighteen (58%) patients had undergone autologous hematopoietic cell transplant (AHCT) with 12 (39%) having undergone AHCT following their diagnosis of PCL. Patients received a median of 2 prior lines of therapy (range 1-9) prior to receiving Dara-based regimens; 15 (42%) were refractory to an immunomodulatory agent (IMiD), 18 (58%) were refractory to a proteasome inhibitor (PI) and 10 (32%) were refractory to both. Twenty-eight (90%) were IMiD exposed, 31 (100%) were PI exposed. Eighteen (58%) were lenalidomide (R) refractory, 5(16%) were pomalidomide (P) refractory, 8 (26%) were carfilzomib (K) refractory and 13(42%) were bortezomib (V) refractory prior to receiving Dara. Four (13%) received Dara + chemotherapy, 8 (26%) received Dara + PI, 11 (35%) received Dara + IMiD, 5 (16%) received Dara + PI + IMiD and 4 (13%) received single agent Dara. Median follow up time was 26 months (m) (95% CI; 13-61) from diagnosis of PCL and 17m (95% CI; 10-23) from initiation of Dara. Overall response rate (ORR) to Dara-based regimens was 65% (20/31 ≥partial response). The median duration of response was 5.5m (95% CI; 5.6-12.7). The median time to first response was 1m (95% CI; 0.57-1.72) and the median time to best response was 1m (95% CI; 0.7-2.5). Since the start of Dara-based treatment, median progression free survival (PFS) was 4m (95% CI; 2-11, Figure 1A) and median overall survival (OS) was 9 m (95% CI; 5-21, Figure 1B). Patients with pPCL had superior PFS (19 m vs. 3m, p=0.0096) and OS (21 vs. 5m, p=0.0068, Figure 1C) following Dara-based treatment compared to those with sPCL, respectively. Being refractory to an IMiD, PI or both when receiving Dara did not affect PFS or OS. Patients with high risk cytogenetics had similar OS as compared to those with standard risk cytogenetics (8m vs.13m, p=0.89) following Dara-based treatment. After performing a landmark survival analysis at 3m, achieving ≥PR to a Dara-based regimen resulted in a trend towards superior OS (13m vs. 9 m, p=0.07, Figure 1D). Patients who underwent AHCT following the diagnosis of PCL had a superior OS (41m vs. 6m, p=0.020) however there were no PFS or OS differences whether Dara-based regimens were given before or after AHCT. Conclusions: Dara-based regimens induce a high ORR in patients with PCL with a rapid time to first response. However, the OS of patients with PCL, particularly sPCL remains dismal. Prospective trials for PCL patients using novel therapeutic strategies are warranted. 1Fernandez de Larrea, et al. Plasma Cell Leukemia: consensus statement on diagnostic requirements, response criteria, and treatment recommendations by the International Myeloma Working Group (IMWG). Leukemia. 2013; 27(4): 780-791. Disclosures Alhaj Moustafa: Acrotech: Consultancy. Kapoor:Janssen: Research Funding; Sanofi: Consultancy, Research Funding; Cellectar: Consultancy; Celgene: Honoraria; Amgen: Research Funding; Takeda: Honoraria, Research Funding; GlaxoSmithKline: Research Funding. Dingli:Millenium: Consultancy; Sanofi-Genzyme: Consultancy; Rigel: Consultancy; Bristol Myers Squibb: Research Funding; Karyopharm Therapeutics: Research Funding; Janssen: Consultancy; Alexion: Consultancy; Apellis: Consultancy. Ailawadhi:Phosplatin: Research Funding; Medimmune: Research Funding; BMS: Research Funding; Cellectar: Research Funding; Pharmacyclics: Research Funding; Janssen: Research Funding; Takeda: Honoraria; Amgen: Research Funding; Celgene: Honoraria.

scholarly journals Cytogenetic and therapeutic characterization of primary plasma cell leukemia: the IFM experience

Leukemia ◽  
2011 ◽  
Vol 26 (1) ◽  
pp. 158-159 ◽  
Author(s):  
H Avet-Loiseau ◽  
M Roussel ◽  
L Campion ◽  
X Leleu ◽  
G Marit ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Primary Plasma Cell Leukemia

Plasma cell leukemia with iga paraproteinemia and hyperviscosity

1988 ◽  
Vol 28 (2) ◽  
pp. 113-115 ◽  
Author(s):  
Michael A. Kosmo ◽  
Robert Peter Gale
Keyword(s):  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia

Primary plasma cell leukemia mimicking a Pancoast tumor

2020 ◽  
Vol 95 (11) ◽  
pp. 1430-1431
Author(s):  
Yorick Sandberg ◽  
Gert T. Verhoeven ◽  
Floor Weerkamp ◽  
Annemiek Broyl ◽  
Jasper Emmering ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Pancoast Tumor ◽  
Primary Plasma Cell Leukemia

Sustained ventricular tachycardia in a thalidomide-treated patient with primary plasma-cell leukemia

2007 ◽  
Vol 4 (12) ◽  
pp. 722-725 ◽  
Author(s):  
Stelvio Ballanti ◽  
Elena Mastrodicasa ◽  
Niccolò Bolli ◽  
Flavia Lotti ◽  
Ilaria Capolsini ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Plasma Cell ◽  
Treated Patient ◽  
Sustained Ventricular Tachycardia ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Primary Plasma Cell Leukemia
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