Plasma cell leukemia

Section editor
	Andrew J. Cowan, MD University of Washington Seattle, WA andrewcowanmd LinkedIn

4 regimens on this page 4 variants on this page

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
Royer et al. 2016	Phase 2

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

Glucocorticoid therapy

• 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

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

Glucocorticoid therapy

• 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

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; IFM. 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
Musto et al. 2013	Phase 2

Targeted therapy

• Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21

Glucocorticoid therapy

• 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

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 2

Preceding treatment

• PAD alternating with VCD

Chemotherapy

• Melphalan (Alkeran) IV once (day not specified) by the following criteria:
  • 65 or younger with CrCl of 30 mL/min/1.73m² or greater: 200 mg/m²
  • 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 EudraCT 2009-016607-33

Maintenance after first-line therapy

Lenalidomide monotherapy

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Regimen

Study	Evidence
Musto et al. 2013	Phase 2

Preceding treatment

• Rd x 8

Targeted therapy

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

28-day cycles

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