Table 1.
Baseline characteristics of patients with stage IIIb AL amyloidosis
| Characteristic | All patients (N = 142) |
|---|---|
| Sex | |
| Male | 99 (70%) |
| Female | 43 (30%) |
| Age, y | 66 (40-90) |
| Cardiopulmonary symptom duration, mo∗ | 6 (1-47) |
| Plasma cell dyscrasia | |
| LC isotype | |
| Kappa | 22 (15%) |
| Lambda | 120 (85%) |
| dFLC, mg/L | 259 (5-9942) |
| BMPC, % | 10 (2-59) |
| Organ function | |
| BNP, pg/mL | 1514 (711-7884) |
| NT-proBNP, pg/mL | 15535 (8503-70000) |
| TnI, ng/mL | 0.399 (0.101-8.915) |
| Alkaline phosphatase, IU/L | 131 (37-2380) |
| Serum albumin, g/dL | 3.6 (1.7-4.6) |
| eGFR, mL per min per 1.73 m2 | 49 (5-112) |
| Urine protein excretion, mg per 24 hours | 435 (0-20000) |
| Dialysis | 10 (7%) |
| Systolic BP, mmHg | 109 (78-172) |
| Diastolic BP, mmHg | 74 (55-102) |
| NYHA class | |
| I | 14 (10%) |
| II | 40 (28%) |
| III | 71 (50%) |
| IV | 17 (12%) |
| 6MWT, m | 274 (0-540) |
| Electrocardiogram | |
| Low voltage | 79 (56%) |
| Atrial fibrillation/flutter | 18 (13%) |
| AV blockade | 29 (20%) |
| RBBB | 21 (15%) |
| LBBB† | 29 (20%) |
| IVCD | 6 (4%) |
| PAC | 12 (8%) |
| PVC | 14 (10%) |
| Echocardiogram | |
| IVSd, mm | 15 (9-24) |
| LVIDd, mm | 41 (25-55) |
| LVPWd, mm | 14 (7-24) |
| LVIDs, mm | 31 (12-47) |
| LVEF, % | 50 (16-70) |
| Diastolic dysfunction | |
| Grade 1 | 17 (14%) |
| Grade 2 | 49 (39%) |
| Grade 3 | 59 (47%) |
| LV mass, g | 230 (88-662) |
| LV mass index, g/m2 | 121 (56-338) |
| LV mass index, g/m | 134 (54-387) |
| Organ involvement (other than cardiac) | |
| Renal | 71 (50%) |
| Hepatic | 29 (20%) |
| GI | 23 (16%) |
| Pulmonary | 5 (4%) |
| ANS | 53 (37%) |
| PNS | 25 (18%) |
| Soft tissue | 34 (24%) |
| Frontline treatment regimen | |
| MDex | 29 (20%) |
| IMiDs | 3 (2%) |
| Bor/Dex | 42 (30%) |
| CyBorD | 54 (38%) |
| Daratumumab‡ | 9 (6%) |
| HDM/SCT | 5 (4%) |
| OHT | 7 (5%) |
Continuous variables are reported as median (range), and categorical variables are reported as number (%). eGFR was calculated using the 2021 CKD-Epi equation.
ANS, autonomic nervous system; AV, atrioventricular; Bor/dex, bortezomib and dexamethasone; BP, blood pressure; CyBorD, cyclophosphamide, bortezomib, and dexamethasone; eGFR, estimated glomerular filtration rate; GI, gastrointestinal; HDM/SCT, high-dose melphalan and autologous stem cell transplantation; IMiDs, immunomodulators (lenalidomide and pomalidomide); IVCD, interventricular conduction delay; IVSd, interventricular septum at end diastole; LBBB, left bundle branch block; LVEF, LV ejection fraction; LVIDd, LV internal diameter at end diastole; LVIDs, LV internal diameter at end systole; LVPWd, LV posterior wall at end diastole; MDex, melphalan and dexamethasone; PAC, premature atrial contraction; PNS, peripheral nervous system; RBBB, right bundle branch block; VC, premature ventricular contraction.
Time between the patient-reported onset of cardiopulmonary symptoms and AL amyloidosis diagnosis.
LBBB includes left anterior fascicular block (n = 23), left posterior fascicular block (n = 3), and complete LBBB (n = 3).
Daratumumab was administered as monotherapy in 4 patients and in combination therapy (CyBorD-Dara [n = 4] and Bor/Dex/Dara [n = 1]) in 5 patients.