Plasma cell leukemia
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Section editor | |
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Andrew J. Cowan, MD University of Washington Seattle, WA andrewcowanmd |
4 regimens on this page
4 variants on this page
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Guidelines
EMN
- 2018: Gavriatopoulou et al. European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias PubMed
IMWG
- 2012: de Larrea et al. Consensus Statement on Diagnostic Requirements, Response Criteria, and Treatment Recommendations by the International Myeloma Working Group (IMWG)
First-line therapy
PAD/VCD
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PAD/VCD: PS-341 (Bortezomib), Adriamycin liposomal (Doxorubicin liposomal), Dexamethasone alternating with Velcade (Bortezomib), Cyclophosphamide, Dexamethasone
Protocol
Study | Evidence |
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Royer et al. 2016 | Phase II |
PAD portion
Targeted therapy
- Bortezomib (Velcade) 1.3 mg/m2 SC once per day on days 1, 4, 8, 11
Chemotherapy
- Pegylated liposomal doxorubicin (Doxil) 30 mg/m2 IV once on day 4
- Dexamethasone (Decadron) 40 mg PO once per day on days 1, 4, 8, 11
21-day cycle for 2 cycles; 1st & 3rd, alternating with VCD
VCD portion
Targeted therapy
- Bortezomib (Velcade) 1.3 mg/m2 SC once per day on days 1, 4, 8, 11
Chemotherapy
- Cyclophosphamide (Cytoxan) 300 mg/m2 PO once per day on days 1 & 8
- Dexamethasone (Decadron) 40 mg PO once per day on days 1, 4, 8, 11
21-day cycles for 2 cycles; 2nd & 4th, alternating with PAD
Subsequent treatment
- Responding patients: High-dose melphalan & autologous hematopoietic stem cell transplant
References
- Royer B, Minvielle S, Diouf M, Roussel M, Karlin L, Hulin C, Arnulf B, Macro M, Cailleres S, Brion A, Brechignac S, Belhadj K, Chretien ML, Wetterwald M, Chaleteix C, Tiab M, Leleu X, Frenzel L, Garderet L, Choquet S, Fuzibet JG, Dauriac C, Forneker LM, Benboubker L, Facon T, Moreau P, Avet-Loiseau H, Marolleau JP. Bortezomib, doxorubicin, cyclophosphamide, dexamethasone induction followed by stem cell transplantation for primary plasma cell leukemia: a prospective phase II study of the Intergroupe Francophone du Myélome. J Clin Oncol. 2016 Jun 20;34(18):2125-32. Epub 2016 Apr 25. link to original article contains verified protocol PubMed EudraCT 2009-016607-33
Lenalidomide & Dexamethasone (Rd)
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Rd: Revlimid (Lenalidomide) & low-dose dexamethasone
RevDex: Revlimid (Lenalidomide) & Dexamethasone
Ld: Lenalidomide & low-dose dexamethasone
LenDex: Lenalidomide & Dexamethasone
Regimen
Study | Evidence |
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Musto et al. 2013 | Phase II |
Targeted therapy
- Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21
- Dexamethasone (Decadron) 40 mg PO once per day on days 1, 8, 15, 22
28-day cycle for 4 cycles
Subsequent treatment
- Responding patients eligible for transplant: HSCT per center protocol
- Responding patients ineligible for transplant received 4 more cycles of Rd, then lenalidomide maintenance
References
- Musto P, Simeon V, Martorelli MC, Petrucci MT, Cascavilla N, Di Raimondo F, Caravita T, Morabito F, Offidani M, Olivieri A, Benevolo G, Mina R, Guariglia R, D'Arena G, Mansueto G, Filardi N, Nobile F, Levi A, Falcone A, Cavalli M, Pietrantuono G, Villani O, Bringhen S, Omedè P, Lerose R, Agnelli L, Todoerti K, Neri A, Boccadoro M, Palumbo A. Lenalidomide and low-dose dexamethasone for newly diagnosed primary plasma cell leukemia. Leukemia. 2014 Jan;28(1):222-5. Epub 2013 Aug 20. link to original article contains verified protocol PubMed
Consolidation after first-line therapy
Melphalan, then auto HSCT
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Regimen
Study | Evidence |
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Royer et al. 2016 | Phase II |
Preceding treatment
Chemotherapy
- Melphalan (Alkeran) 200 mg/m2 IV once
- Patients older than 65 years or with CrCl less than 30 mL/min/1.73m2: 140 mg/m2
References
- Royer B, Minvielle S, Diouf M, Roussel M, Karlin L, Hulin C, Arnulf B, Macro M, Cailleres S, Brion A, Brechignac S, Belhadj K, Chretien ML, Wetterwald M, Chaleteix C, Tiab M, Leleu X, Frenzel L, Garderet L, Choquet S, Fuzibet JG, Dauriac C, Forneker LM, Benboubker L, Facon T, Moreau P, Avet-Loiseau H, Marolleau JP. Bortezomib, Doxorubicin, Cyclophosphamide, Dexamethasone Induction Followed by Stem Cell Transplantation for Primary Plasma Cell Leukemia: A Prospective Phase II Study of the Intergroupe Francophone du Myélome. J Clin Oncol. 2016 Jun 20;34(18):2125-32. Epub 2016 Apr 25. link to original article contains protocol PubMed EudraCT 2009-016607-33
Maintenance after first-line therapy
Lenalidomide monotherapy
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Regimen
Study | Evidence |
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Musto et al. 2013 | Phase II |
Preceding treatment
- Rd x 8
Targeted therapy
- Lenalidomide (Revlimid) 10 mg PO once per day on days 1 to 21
28-day cycles
References
- Musto P, Simeon V, Martorelli MC, Petrucci MT, Cascavilla N, Di Raimondo F, Caravita T, Morabito F, Offidani M, Olivieri A, Benevolo G, Mina R, Guariglia R, D'Arena G, Mansueto G, Filardi N, Nobile F, Levi A, Falcone A, Cavalli M, Pietrantuono G, Villani O, Bringhen S, Omedè P, Lerose R, Agnelli L, Todoerti K, Neri A, Boccadoro M, Palumbo A. Lenalidomide and low-dose dexamethasone for newly diagnosed primary plasma cell leukemia. Leukemia. 2014 Jan;28(1):222-5. Epub 2013 Aug 20. link to original article contains verified protocol PubMed