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

1. 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

1. 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
2. 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

1. 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

1. 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

1. 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

1. 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

1. 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

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

1. 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
2. Kumar SK, Hayman SR, Buadi FK, Roy V, Lacy MQ, Gertz MA, Allred J, Laumann KM, Bergsagel LP, Dingli D, Mikhael JR, Reeder CB, Stewart AK, Zeldenrust SR, Greipp PR, Lust JA, Fonseca R, Russell SJ, Rajkumar SV, Dispenzieri A. Lenalidomide, cyclophosphamide, and dexamethasone (CRd) for light-chain amyloidosis: long-term results from a phase 2 trial. Blood. 2012 May 24;119(21):4860-7. doi:10.1182/blood-2012-01-407791. Epub 2012 Apr 13. PubMed PMID: 22504925; PubMedCentral PMCID: PMC3418771.link to original article contains protocol PubMed

CTD

CTD: Cyclophosphamide, Thalidomide, Dexamethasone

Regimen

• Cyclophosphamide (Cytoxan) 500 mg PO once weekly
• Thalidomide (Thalomid) 100 mg PO daily, increased to 200 mg daily after 4 weeks if well tolerated
• Dexamethasone (Decadron) 40 mg PO daily, days 1-4 and 9-12

21-day cycle for 4-12 cycles or until hematologic complete response

Risk attenuated regimen

For elderly patients (age > 70 years), NYHA heart failure > class II, and those with significant fluid retention

• Cyclophosphamide (Cytoxan) 500 mg PO daily, days 1, 8, and 15
• Thalidomide (Thalomid) 50 mg PO daily, increased by 50 mg at 4-week intervals as tolerated to maximum dose of 200 mg daily
• Dexamethasone (Decadron) 20 mg PO daily, days 1-4 and 9-12

28-day cycle for 4-12 cycles or until hematologic complete response

Supportive medications: Antimicrobial and thromboprophylaxis per provider preference

References

1. Wechalekar AD, Goodman HJ, Lachmann HJ, Offer M, Hawkins PN, Gillmore JD. Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis. Blood. 2007 Jan 15;109(2):457-64. Epub 2006 Sep 21. PubMed PMID: 16990593.

link to original article contains protocol PubMed

Relapsed/Refractory Disease

Pomalidomide

Regimen

• Pomalidomide (Pomalyst) 2 mg PO daily for 28 days
• Dexamethasone (Decadron) 40 mg PO once weekly

28-day cycle for 2-9 cycles or until hematologic complete response

Supportive medications:

• Aspirin 325 mg PO daily for thromboprophylaxis

References

1. Dispenzieri A, Buadi F, Laumann K, LaPlant B, Hayman SR, Kumar SK, Dingli D, Zeldenrust SR, Mikhael JR, Hall R, Rajkumar SV, Reeder C, Fonseca R, Bergsagel PL, Stewart AK, Roy V, Witzig TE, Lust JA, Russell SJ, Gertz MA, Lacy MQ. Activity of pomalidomide in patients with immunoglobulin light-chain amyloidosis. Blood. 2012 Jun 7;119(23):5397-404. doi: 10.1182/blood-2012-02-413161. Epub 2012 Apr 4. PubMed PMID: 22493299; PubMed Central PMCID: PMC3369677.

link to original article contains protocol PubMed