Light-chain (AL) amyloidosis
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First-Line Therapy (including transplant ineligible)
Lenalidomide
Regimen
- Lenalidomide (Revlimid) 15 mg PO daily on days 1-21
- Dexamethasone (Decadron) 10-20 mg/day from days 1 to 4, 9 to 12, and 17 to 20 every other cycle, added if no response to lenalidomide after the first 3 cycles
28-day cycles x 6 cycles
Supportive medications:
- Aspirin 325 mg PO once daily
- Pantoprazole (Protonix) 40 mg PO once daily
References
- Sanchorawala V, Wright DG, Rosenzweig M, Finn KT, Fennessey S, Zeldis JB, Skinner M, Seldin DC. Lenalidomide and dexamethasone in the treatment of AL amyloidosis: results of a phase 2 trial. Blood. 2007 Jan 15;109(2):492-6. Epub 2006 Sep 7. PubMed PMID: 16960148.
link to original article contains protocol PubMed
Bortezomib
Regimen
- Bortezomib (Velcade) 1.3 mg/m2 IV on days 1, 8, 15, 22
- Dexamethasone (Decadron) 40 mg PO/IV on days 1, 8, 15, 22; can decrease dose to 20 mg PO/IV days 1, 8, 15, 22 for patients with edema due to CHF or nephrotic syndrome, patients with cardiac involvement, and age over 70 years.
35-day cycle x 8 cycles or until hematologic complete response
Supportive medications:
- Pantoprazole (Protonix) 40 mg PO every day
- Acyclovir 400 mg PO BID
References
- Reece DE, Hegenbart U, Sanchorawala V, et al. Efficacy and safety of once-weekly and twice-weekly bortezomib in patients with relapsed systemic AL amyloidosis: results of a phase 1/2 study. Blood;118(4):865-73.
link to original article contains protocol PubMed
- Reece DE, Sanchorawala V, Hegenbart U, et al. Weekly and twice-weekly bortezomib in patients with systemic AL amyloidosis: results of a phase 1 dose-escalation study. Blood. 2009;114:1489-1497.
link to original article contains protocol PubMed
Melphalan
Regimen
- Melphalan (Alkeran) 0.22 mg/kg/day for 4 days
- Dexamethasone (Decadron) 40 mg/day for 4 days
28-day cycle for 6-9 cycles
Supportive medications:
- Pantoprazole (Protonix) 40 mg PO every day
References
- Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation.
Palladini G, Perfetti V, Obici L, Caccialanza R , Semino A, Adami F, Cavallero G, Rustichelli R, Virga G, Merlini G. Blood. 2004;103:2936–2938 link to original article contains protocol PubMed
VMD
VMD: Velcade, Melphalan, Dexamethasone
Regimen
- Melphalan (Alkeran) 9 mg/m2 PO days 1-4; 6 mg/m2 if serum creatinine > 2.5 mg/dL,
- Bortezomib (Velcade) 1.3 mg/m2 IV days 1, 8, 15, 22; 1.0 mg/m2 if patient has peripheral neuropathy at baseline
- Dexamethasone (Decadron) 40 mg PO/IV days 1, 8, 15, 22; 20 mg if > 70 years of age, peripheral edema, or CHF
4-6 week cycle, maximum of 20 cycles
Supportive medications:
- Pantoprazole (Protonix) 40 mg PO every day
- Acyclovir 400 mg PO BID
References
- Zonder JA, Sanchorawala V, Snyder RM, Matous J, Terebelo H, Janakiraman N, Mapara MY, Lalo S, Tageja N, Webb C, Monsma D, Sellers C, Abrams J, Gasparetto C. Melphalan and Dexamethasone Plus Bortezomib Induces Hematologic and Organ Responses in AL Amyloidosis with Tolerable Neurotoxicity. Blood (ASH Annual Meeting Abstracts), Nov 2009; 114: 746.
CyBorD
CyBorD: Cyclophosphamide, Bortezomib, Dexamethasone
Regimen
- Cyclophosphamide (Cytoxan) 300 mg/m2 PO weekly
- Bortezomib (Velcade) 1.5 mg/m2 IV weekly OR 1.3 mg/m2 days 1, 4, 8 and 11
- Dexamethasone (Decadron) 40 mg PO weekly
4-week cycle, 2 to 6 cycles or until hematologic response
Supportive medications:
- Pantoprazole (Protonix) 40 mg PO every day
- Acyclovir 400 mg PO BID
References
- Mikhael JR, Schuster SR, Jimenez-Zepeda VH, Bello N, Spong J, Reeder CB, Stewart AK, Bergsagel PL, Fonseca R. Cyclophosphamide-bortezomib-dexamethasone (CyBorD) produces rapid and complete hematologic response in patients with AL amyloidosis. Blood. 2012 May 10;119(19):4391-4. doi:10.1182/blood-2011-11-390930. Epub 2012 Feb 13. PubMed PMID: 22331188; PubMed Central PMCID: PMC3557400.
link to original article contains protocol PubMed
MRD
MRD: Melphaln, Revlimid, Dexamethasone
Regimen
- Melphalan (Alkeran) 5 mg/m2 PO days 1-4
- Lenalidomide (Revlimid) 10 mg PO daily for days 1-21
- Dexamethasone (Decadron) 40 mg PO weekly
28-day cycle for 3-6 cycles or until hematologic complete response
Supportive medications:
- Aspirin 325 mg PO daily to decrease risk of venous thromboembolism associated with lenalidomide
- Pantoprazole (Protonix) 40 mg PO daily
References
- Sanchorawala V, Patel JM, Sloan JM, Shelton AC, Zeldis JB, Seldin DC. Melphalan, lenalidomide and dexamethasone for the treatment of AL amyloidosis: results of a phase II trial. Haematologica. 2012 Nov 9. [Epub ahead of print] PubMed PMID: 23144200.
link to original article contains protocol PubMed
RdC
RdC: Revlimid, dexamethasone, Cyclophosphamide
Regimen
- Lenalidomide (Revlimid) 15 mg PO daily, days 1-21
- Cyclophosphamide (Cytoxan) 100 mg PO daily, days 1-10
- Dexamethasone (Decadron) 20 mg PO daily, days 1-4
28-day cycle for 6-12 cycles or until hematologic complete response
Supportive medications:
- Aspirin 100 mg PO daily for thromboprophylaxis
- Pantoprazole (Protonix) 40 mg PO daily
- Trimethoprim-sulfamethoxazole (Bactrim) Dose not listed
- Valaciclovir (Valcyte) Dose not listed
References
- Kastritis E, Terpos E, Roussou M, Gavriatopoulou M, Pamboukas C, Boletis I, Marinaki S, Apostolou T, Nikitas N, Gkortzolidis G, Michalis E, Delimpasi S, Dimopoulos MA. A phase 1/2 study of lenalidomide with low-dose oral cyclophosphamide and low-dose dexamethasone (RdC) in AL amyloidosis. Blood. 2012 Jun 7;119(23):5384-90. doi: 10.1182/blood-2011-12-396903. Epub 2012 Apr 18. PubMed PMID: 22517904.
link to original article contains protocol PubMed
Relapsed/Refractory Disease
CRD
CRD: Cyclophosphamide, Revlimid, Dexamethasone
Regimen
- Cyclophosphamide (Cytoxan) 500 mg PO days 1, 8, 15
- Lenalidomide (Revlimid) 15 mg PO days 1-21
- Dexamethasone (Decadron) 40 mg PO weekly
28-day cycle for 4 cycles or until hematologic complete response
Supportive medications:
- Aspirin 100 mg PO daily for thromboprophylaxis
References
- Palladini G, Russo P, Milani P, Foli A, Lavatelli F, Nuvolone M, Perlini S, Merlini G. A phase II trial of cyclophosphamide, lenalidomide and dexamethasone in previously treated patients with AL amyloidosis. Haematologica. 2013 Mar;98(3):433-6. doi: 10.3324/haematol.2012.073593. Epub 2012 Sep 14. PubMed PMID: 22983583.
link to original article contains protocol PubMed