Plasma Cell Leukemia: A retrospective review of cases at Monter Cancer Center/Northwell Health Cancer Institute 2014-2019 1Donald
Christina Cotte1, David Chitty2, & Monique Hartley-Brown1,2
and Barbara Zucker School of Medicine at Hofstra/Northwell, 2Northwell Health Cancer Institute, Monter Cancer Center, Lake Success, New York, USA
Background
Results
Discussion
Plasma cell leukemia (PCL) is an aggressive relative of MM with acute overall survival(OS) of 13 months that can be primary or secondary to MM.1,2 The defining criteria for PCL is in flux (20% circulating plasma cells vs. >5%).1,3 With changing diagnostic criteria, difficulty studying the disease due to its fulminance and rarity, and a lack of clear guidance on treatment, it is crucial to explore the impact of novel treatment options on the survival of patients with PCL.1,2,3,4
Hypothesis Our goal is to gather data about the treatment of PCL so regimens associated with better OS can be elucidated. We hypothesize that patients diagnosed with PCL continue to have improved outcomes when treated with newer FDA approved therapies2, such as Dara2,5,6, compared with conventional therapy.
Methods • Retrospective chart analysis of patients with primary and secondary PCL (IMWG diagnostic criteria) in the Northwell Health system from 20142019.7,8,9 • Demographics, diagnoses, cytogenetics, FISH, laboratory results, and treatment regimens were collected. • Survival outcome analysis using time to relapse from primary treatment and OS performed.
Figure 1: Overall Survival (OS) vs. Treatment for pPCL & sPCL – OS in pPCL was longer than sPCL (OS ranging from 3-20 mo.), with two pPCL patients alive at endpoint. 6/8 patients received a proteasome inhibitor, IMiD, and steroid. 2/8 patients received Dara-d.
Key: CRd (Carfilzomib, Lenalidomide, Dexamethasone), ASCT (autologous stem cell transplant), Dara-d (Daratumumab, Dexamethasone), VPd (Bortezomib, Pomalidomide, Dexamethasone), HiDEX (dexamethasone), CyBorD (Cyclophosphamide, Bortezomib, Dexamethasone), DCEP (Dexamethasone, Cyclophosphamide, Etoposide, Cisplatin), VTD-PACE (Dexamethasone, Thalidomide, Cisplatin, Doxorubicin), IT MTX (intrathecal MTX), NR (no response), PD (progression of disease), CR(complete response), VGPR (very good partial response), IMiD (immunomodulatory imide drugs), pPCL (primary PCL), sPCL (secondary PCL)
Based on the SEER database, the OS of pPCL has been increasing between 1973 and 2009 from 5 months up to 12 months.11 All pPCL patients included in this study had OS > 12 mo., including 2 patients in remission > 8 years. Median OS for patients with sPCL has historically been shorter than pPCL (median OS = 1.3 mo., range 2-7 mo.).2,10,11 Evidence of improving survival with use of novel agents has been eluded to10 and is supported by our data. It also appears that the OS has prolonged to a greater extend for pPCL compared to sPCL. Our patients were > 60 y/o except 1 outlier. There was no clear distinction of pPCL presenting younger than sPCL in this cohort, as has been concluded previously.2 Transformation from MM to sPCL also varied dramatically and did not always fall within 2022 months.2 Compared to a prior report2, use of Daratumumab (+VCd or VPd) did not have a dramatic effect on survival in this cohort, but these patients did not have similar cytogenetics and these therapies were not used first line. Treatment regimens for this rare condition are not uniform as there is no clear standard of treatment, even though the novel therapy (proteasome inhibitor + IMiD +/- steroid) + ASCT is favored. Further analysis of laboratories and cytogenetics will be performed
Future Direction need.2,4,7
PCL is an aggressive malignancy and an area of unmet Due to its fulminant course and the lack of prospective clinical trials in PCL, the optimal therapeutic approach remains controversial.2 We propose that multi-institutional meta-analyses and prospective studies in PCL will be important in determining better treatment. In addition, single institutional studies with a greater time frame will be helpful in delineating the overall benefit of novel therapies on PCL treatment compared to conventional chemotherapy, which is no longer used. More cases of diverse Dara use in the treatment of PCL also need to be documented to better understand its impact.
Resources 1. 2. 3. 4. 5.
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