Characteristics of 36 patients with AL amyloidosis and nonlymphoplasmacytic LPDs
| Characteristic . | Systemic AL amyloidosis, 21 patients (No. [IQR or %]) . | Localized AL amyloidosis, 15 patients (No. [IQR or %]) . |
|---|---|---|
| Age, y | 63 (63-69) | 70 (64-74) |
| Male sex | 15 (71) | 9 (60) |
| MZL | 9 (43) | 10 (67) |
| Extranodal/nodal/disseminated/NOS | 4 (19)/0/1 (5)/4 (19) | 7 (46)/1 (7)/1 (7)/1 (7) |
| Non-MZL | 12 (57) | 5 (33) |
| Low-grade B-cell lymphoma NOS | 5 (24) | 2 (13) |
| DLBCL/CLL-SLL/other diagnosis* | 2 (10)/2 (10)/3 (13) | 2 (13)/1 (7)/0 |
| Organ involvement in systemic AL amyloidosis | NA | |
| Heart/kidney/liver/soft tissues/ANS/PNS | 12 (57)/8 (38)/1 (5)/4 (19)/3 (13)/3 (13) | |
| Site of localized AL amyloidosis | NA | |
| Nodular pulmonary/lymph nodes | 7 (46)/3 (10) | |
| Skin/tracheobronchial/bladder/nodular GI | 2 (13)/1 (7)/1 (7)/1 (7) | |
| NT-proBNP, ng/L | 1113 (558-6979) | 152.28 (47-265) |
| Proteinuria, g/24 h | 1.21 (0.10-5.00) | 0.07 (0.06-0.09) |
| Cardiac stage I/II/IIIa/IIIb† | 8 (32)/11 (44)/4 (16)/2 (8) | NA |
| Renal stage I/II/III‡ | 15 (60)/8 (32)/2 (8) | NA |
| Ann Arbor stage I/II/III/IV | 2 (10)/1 (5)/1 (5)/17 (80) | 8 (53)/0/2 (13)/5 (34) |
| Monoclonal component at serum and/or urine immunofixation | 20 (95) | 8 (53) |
| Kappa: lambda ratio | 4 (20): 16 (80) | 4 (50): 4 (50) |
| Monoclonal component | ||
| IgGλ/IgGκ/IgAλ/IgAκ | 1 (5)/6 (28)/1 (5)/1 (5) | 1 (7)/3 (20)/0/0 |
| IgMλ/IgMκ/FLC λ/FLC κ | 2 (10)/2 (10)/5 (24)/0 | 2 (13)/0/1 (7)/1 (7) |
| Free light chain only: complete monoclonal component | 6 (29): 14 (67) | 2 (25): 6 (75) |
| Positive fat pad aspirate (Congo red) | 17 (80) | 0 |
| Abnormal FLCR | 15 (71) | 5 (33) |
| B symptoms | 3 (13) | 1 (7) |
| HCV/HBV infection | 2 (10)/3 (13) | 0/3 (20) |
| Treatment of lymphoma | 14 (66) | 10 (67) |
| R-CHOP/R-CVP/radiotherapy | 7 (33)/2 (10)/0 | 5 (34)/1 (7)/2 (13) |
| Alkylating agents/other treatment§ | 3 (13)/2 (10) | 0/2 (13) |
| Characteristic . | Systemic AL amyloidosis, 21 patients (No. [IQR or %]) . | Localized AL amyloidosis, 15 patients (No. [IQR or %]) . |
|---|---|---|
| Age, y | 63 (63-69) | 70 (64-74) |
| Male sex | 15 (71) | 9 (60) |
| MZL | 9 (43) | 10 (67) |
| Extranodal/nodal/disseminated/NOS | 4 (19)/0/1 (5)/4 (19) | 7 (46)/1 (7)/1 (7)/1 (7) |
| Non-MZL | 12 (57) | 5 (33) |
| Low-grade B-cell lymphoma NOS | 5 (24) | 2 (13) |
| DLBCL/CLL-SLL/other diagnosis* | 2 (10)/2 (10)/3 (13) | 2 (13)/1 (7)/0 |
| Organ involvement in systemic AL amyloidosis | NA | |
| Heart/kidney/liver/soft tissues/ANS/PNS | 12 (57)/8 (38)/1 (5)/4 (19)/3 (13)/3 (13) | |
| Site of localized AL amyloidosis | NA | |
| Nodular pulmonary/lymph nodes | 7 (46)/3 (10) | |
| Skin/tracheobronchial/bladder/nodular GI | 2 (13)/1 (7)/1 (7)/1 (7) | |
| NT-proBNP, ng/L | 1113 (558-6979) | 152.28 (47-265) |
| Proteinuria, g/24 h | 1.21 (0.10-5.00) | 0.07 (0.06-0.09) |
| Cardiac stage I/II/IIIa/IIIb† | 8 (32)/11 (44)/4 (16)/2 (8) | NA |
| Renal stage I/II/III‡ | 15 (60)/8 (32)/2 (8) | NA |
| Ann Arbor stage I/II/III/IV | 2 (10)/1 (5)/1 (5)/17 (80) | 8 (53)/0/2 (13)/5 (34) |
| Monoclonal component at serum and/or urine immunofixation | 20 (95) | 8 (53) |
| Kappa: lambda ratio | 4 (20): 16 (80) | 4 (50): 4 (50) |
| Monoclonal component | ||
| IgGλ/IgGκ/IgAλ/IgAκ | 1 (5)/6 (28)/1 (5)/1 (5) | 1 (7)/3 (20)/0/0 |
| IgMλ/IgMκ/FLC λ/FLC κ | 2 (10)/2 (10)/5 (24)/0 | 2 (13)/0/1 (7)/1 (7) |
| Free light chain only: complete monoclonal component | 6 (29): 14 (67) | 2 (25): 6 (75) |
| Positive fat pad aspirate (Congo red) | 17 (80) | 0 |
| Abnormal FLCR | 15 (71) | 5 (33) |
| B symptoms | 3 (13) | 1 (7) |
| HCV/HBV infection | 2 (10)/3 (13) | 0/3 (20) |
| Treatment of lymphoma | 14 (66) | 10 (67) |
| R-CHOP/R-CVP/radiotherapy | 7 (33)/2 (10)/0 | 5 (34)/1 (7)/2 (13) |
| Alkylating agents/other treatment§ | 3 (13)/2 (10) | 0/2 (13) |
Variables from patients with systemic and localized AL amyloidosis were tested for statistical significance if applicable. ANS, autonomic nervous system; CLL-SLL, chronic lymphocytic leukemia–small lymphocytic lymphoma; DLBCL, diffuse large B-cell lymphoma; FLCR, free light chain ratio; HBV, hepatitis B virus; HCV, hepatitis C virus; GI, gastrointestinal; NA, not applicable; NOS, not otherwise specified; NT-proBNP, N-terminal pro–B-type natriuretic peptide; PNS, peripheral nervous system; R-CHOP, rituximab, cyclophosphamide, adriamycin, vincristine, prednisone; R-CVP, rituximab, cyclophosphamide, vincristine, prednisone.
Other diagnosis: 1 follicular lymphoma, 1 hairy cell leukemia, and 1 Hodgkin lymphoma.
Cardiac stage: Based on NT-proBNP (cutoff, 332 ng/L) and cardiac troponin I (cutoff, 0.1 ng/mL). Stage I patients have both markers below the cutoffs; stage II patients, only one marker above the cutoffs; and stage III patients, both markers above the cutoffs. NT-proBNP >8500 ng/L divided stage III patients into stage IIIa and IIIb cases.
Renal stage: Stage I, both proteinuria ≤5 g/24 hours and estimated glomerular filtration rate (eGFR) ≥50 mL/min per 1.73 m2; stage II, either proteinuria >5 g/24 hours or eGFR <50 mL/min per 1.73 m2; stage III, both proteinuria >5 g/24 hours and eGFR <50 mL/min per 1.73 m2.
Other treatment: 1 fludarabine and cyclophosphamide and rituximab, and 1 cladribine in patients with systemic AL amyloidosis and 1 rituximab in monotherapy, 1 chlorambucil in patients with localized AL amyloidosis