Plasma cell leukemia

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Section editor
	Andrew J. Cowan, MD Seattle, WA LinkedIn

4 regimens on this page 4 variants on this page

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

Regimen

Study	Evidence
Royer et al. 2016	Phase II

Chemotherapy, PAD portion

• Bortezomib (Velcade) 1.3 mg/m² SC once per day on days 1, 4, 8, 11
• 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

Two 21-day cycles (1 & 3), alternating with VCD

Chemotherapy, VCD portion

• Bortezomib (Velcade) 1.3 mg/m² SC once per day on days 1, 4, 8, 11
• 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

Two 21-day cycles (2 & 4), alternating with PAD

Responding patients proceeded to undergo high-dose melphalan & autologous hematopoietic stem cell transplant.

References

1. 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

Rd

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Rd: Revlimid (Lenalidomide) & low-dose dexamethasone
RevDex: Revlimid (Lenalidomide) & Dexamethasone
Ld: Lenalidomide & low-dose dexamethasone
Len-Dex: Lenalidomide & Dexamethasone

Regimen

Study	Evidence
Musto et al. 2013	Phase II

Chemotherapy

• 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

Responding patients eligible for transplant proceeded to SCT per center protocol. Responding patients ineligible for transplant received 4 more cycles of Rd, followed by lenalidomide maintenance.

References

1. 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
Royer et al. 2016	Phase II

Preceding treatment

• PAD alternating with VCD

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

1. 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

Maintenance after first-line therapy

Lenalidomide monotherapy

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Regimen

Study	Evidence
Musto et al. 2013	Phase II

Preceding treatment

• Rd x 8

Chemotherapy

• Lenalidomide (Revlimid) 10 mg PO once per day on days 1 to 21

28-day cycles until progression

References

1. 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