Table 3.

Available Treatments for AL Amyloidosis

Regimen	No. of Patients	No. of Patients Previously Treated	Patients With Heart Involvement (%)a	HemR/CR (%)	Organ Response (%)	TRM (%)	SAE Grade ≥ 3	Reference
Melphalan-prednisone-colchicine	50		76	NR	20	NR	8	Skinner et al53
Melphalan-prednisone	148b		NRc	28d	18	NR	5e	Kyle et al54
Dexamethasonef	23	13	39	NR	30	0	NR	Palladini et al55
Dexamethasone	25		68	40/16	12	8	NR	Gertz et al56
Dexamethasone	19	19	63	53/10	16	5	NR	Gertz et al57
Dexamethasone + maintenance IFN-α	87	14	50	33/15	45	7	51/67g	Dhodapkar et al58
HDM/SCT (single-center data)	312		43	58h/23	26	13	NR	Skinner et al59
HDM/SCT (single-center data)	171		49	68/NR	NR	12	NR	Gertz et al60
Melphalan-dexamethasone	46		70	67/33	48	4	11	Palladini et al61
Melphalan-dexamethasone	43		46	68/32	39	2	16	Jaccard et al62
Thalidomide-dexamethasone	31	31	38	48/19	26	0	65	Palladini et al63
Cyclophosphamide-thalidomide-dexamethasone	75	44	59	74/21	27	4	32	Wechalekar et al64
Lenalidomide ± dexamethasone	22	13	64	41/NR	23	18	86	Dispenzieri et al65
Lenalidomide ± dexamethasone	34	31	38	47/21	21	3	35	Sanchorawala et al66
Bortezomib	49	49	57	67/36	35	0	50/79i	Reece et al67
Bortezomib + dexamethasone	94j	76	73	71/25	30	0	29	Kastritis et al42
Pomalidomide + dexamethasone	25	25	80	47/10k	10	NR	56	Dispenzieri et al68

Abbreviations: AL, immunoglobin light chain amyloidosis; HemR, hematologic response; CR, complete response; TRM, treatment-related mortality; SAE, severe adverse event; NR, not reported; IFN-α, interferon alfa; HDM, high-dose melphalan; SCT, stem-cell transplantation.

^aCriteria for heart involvement were heterogeneous before the adoption of the consensus criteria in 2005.⁴⁷

^bIncluding 71 patients treated with melphalan-prednisone-colchicine.

^c Twenty percent of patients had dominant heart involvement.

^dComposite response including, in addition to disappearance of or a reduction of at least 50% in the serum or urine monoclonal protein, an increase ≥ 1 g in serum albumin value, and a reduction ≥ 50% in urinary protein excretion.

^eIncludes development of myelodysplasia in seven patients and acute leukemia in one patient.

^fDose of 40 mg on days 1 to 4, every 21 days.

^gInduction phase/maintenance phase.

^hReported as noncomplete hematologic response.

ⁱAdministration of bortezomib once weekly or twice weekly.

^jEleven percent of the patients did not receive dexamethasone.

^k Very good partial responses.