Comparison of Patients With Primary Systemic Amyloidosis, by Duration of Survival
| Factor . | Nonsurvivors (<10 yr) Seen ≤6 Mo of Diagnosis (n = 599) . | Survivors ≥10 Yr . | P Value . | |
|---|---|---|---|---|
| Total Group (n = 30) . | Seen ≤6 Mo of Diagnosis (n = 19) . | |||
| Age, median (yr) | 63 | 54 | 54 | .001 |
| Sex, male (%) | 65 | 53 | 47 | .12 |
| Weight loss (%) | 49 | 31 | 33 | .18 |
| Liver palpable (%) | 34 | 31 | 33 | .94 |
| Macroglossia (%) | 19 | 21 | 12 | .75 |
| Hemoglobin <10 g/dL (%) | 12 | 7 | 6 | .71 |
| Platelets >500 × 109/L (%) | 10 | 24 | 33 | .01 |
| Alkaline phosphatase ≥500 U/L (%) | 8 | 7 | 0 | .38 |
| Creatinine ≥2.0 mg/dL (%) | 22 | 14 | 12 | .55 |
| Bone marrow plasma cells ≥20% (%) | 21 | 4 | 6 | .21 |
| Serum albumin <3 g/dL (%) | 59 | 37 | 37 | .14 |
| Serum monoclonal protein (%) | 70 | 64 | 59 | .33 |
| Urine positive (%) | 74 | 62 | 69 | .57 |
| Serum or urine monoclonal protein present (%) | 89 | 86 | 88 | 1.00 |
| Interventricular septal thickness ≥15 mm* (%) | 43 | 36 | 33 | .51 |
| Carpal tunnel syndrome (%) | 25 | 20 | 21 | 1.00 |
| Nephrotic range proteinuria (%) | 34 | 33 | 32 | .74 |
| CHF (%) | 24 | 7 | 11 | .08 |
| Peripheral neuropathy (%) | 18 | 7 | 5 | .22 |
| Orthostatic hypotension (%) | 16 | 13 | 16 | 1.00 |
| Bone marrow biopsy positive for amyloid deposits (%) | 46 | 64 | 61 | .37 |
| Rectal biopsy positive for amyloid deposits (%) | 75 | 65 | 83 | .75 |
| Therapy | ||||
| ≤30 d of diagnosis (%) | 64 | 33 | 44 | .09 |
| ≤180 d of diagnosis (%) | 94 | 52 | 72 | .01 |
| Factor . | Nonsurvivors (<10 yr) Seen ≤6 Mo of Diagnosis (n = 599) . | Survivors ≥10 Yr . | P Value . | |
|---|---|---|---|---|
| Total Group (n = 30) . | Seen ≤6 Mo of Diagnosis (n = 19) . | |||
| Age, median (yr) | 63 | 54 | 54 | .001 |
| Sex, male (%) | 65 | 53 | 47 | .12 |
| Weight loss (%) | 49 | 31 | 33 | .18 |
| Liver palpable (%) | 34 | 31 | 33 | .94 |
| Macroglossia (%) | 19 | 21 | 12 | .75 |
| Hemoglobin <10 g/dL (%) | 12 | 7 | 6 | .71 |
| Platelets >500 × 109/L (%) | 10 | 24 | 33 | .01 |
| Alkaline phosphatase ≥500 U/L (%) | 8 | 7 | 0 | .38 |
| Creatinine ≥2.0 mg/dL (%) | 22 | 14 | 12 | .55 |
| Bone marrow plasma cells ≥20% (%) | 21 | 4 | 6 | .21 |
| Serum albumin <3 g/dL (%) | 59 | 37 | 37 | .14 |
| Serum monoclonal protein (%) | 70 | 64 | 59 | .33 |
| Urine positive (%) | 74 | 62 | 69 | .57 |
| Serum or urine monoclonal protein present (%) | 89 | 86 | 88 | 1.00 |
| Interventricular septal thickness ≥15 mm* (%) | 43 | 36 | 33 | .51 |
| Carpal tunnel syndrome (%) | 25 | 20 | 21 | 1.00 |
| Nephrotic range proteinuria (%) | 34 | 33 | 32 | .74 |
| CHF (%) | 24 | 7 | 11 | .08 |
| Peripheral neuropathy (%) | 18 | 7 | 5 | .22 |
| Orthostatic hypotension (%) | 16 | 13 | 16 | 1.00 |
| Bone marrow biopsy positive for amyloid deposits (%) | 46 | 64 | 61 | .37 |
| Rectal biopsy positive for amyloid deposits (%) | 75 | 65 | 83 | .75 |
| Therapy | ||||
| ≤30 d of diagnosis (%) | 64 | 33 | 44 | .09 |
| ≤180 d of diagnosis (%) | 94 | 52 | 72 | .01 |
Abbreviation: CHF, congestive heart failure.
Determined with echocardiography.