Patients seen between June 1, 2002, and August 1, 2005, for assessment of systemic amyloidosis
Feature . | Data . |
|---|---|
| No. patients | 178 |
| No. men/no. women | 110/68 |
| Median age, y (range) | 60 (34-83) |
| Diagnoses, no. | 170 |
| AL | 150 |
| ATTR | 7 |
| Both MG and hereditary variant identified | 3 |
| AA | 1 |
| Senile cardiac | 2 |
| Plasmacytoma | 2 |
| Other | 5 |
| Asymptomatic patients, no. | 5 |
| ATTR | 2 |
| Localized AL in plasmacytomas | 2 |
| Localized with cancer | 1 |
| No. patients with symptoms of organ involvement | 165 |
| Dominant organ involvement, no. (%) | |
| Cardiac | 69 (42) |
| Renal | 50 (30) |
| Other | 46 (28) |
| Subsets screened for hereditary variants, no. | |
| Patients (% of total) | 54 (30) |
| African-American (no. with MG/no. without MG) | 20 (16/4) |
| Peripheral neuropathy (no. with MG/no. without MG) | 16 (11/5) |
| Renal without marrow amyloid (no. with MG/no. without MG) | 7 (7/0) |
| Other (no. with MG/no. without MG) | 11 (1/10) |
Feature . | Data . |
|---|---|
| No. patients | 178 |
| No. men/no. women | 110/68 |
| Median age, y (range) | 60 (34-83) |
| Diagnoses, no. | 170 |
| AL | 150 |
| ATTR | 7 |
| Both MG and hereditary variant identified | 3 |
| AA | 1 |
| Senile cardiac | 2 |
| Plasmacytoma | 2 |
| Other | 5 |
| Asymptomatic patients, no. | 5 |
| ATTR | 2 |
| Localized AL in plasmacytomas | 2 |
| Localized with cancer | 1 |
| No. patients with symptoms of organ involvement | 165 |
| Dominant organ involvement, no. (%) | |
| Cardiac | 69 (42) |
| Renal | 50 (30) |
| Other | 46 (28) |
| Subsets screened for hereditary variants, no. | |
| Patients (% of total) | 54 (30) |
| African-American (no. with MG/no. without MG) | 20 (16/4) |
| Peripheral neuropathy (no. with MG/no. without MG) | 16 (11/5) |
| Renal without marrow amyloid (no. with MG/no. without MG) | 7 (7/0) |
| Other (no. with MG/no. without MG) | 11 (1/10) |
There were 3 patients with both a monoclonal gammopathy and an hereditary variant: a 46-year-old man with soft-tissue, nodal, and cardiac amyloid (TTR-negative by immunohistochemical staining [IHC]) with hereditary variant V1222I TTR and free λ of 28 mg/dL with 28% λ-restricted plasma cells; a 59-year-old man with peripheral neuropathy (TTR-positive by IHC) with hereditary variant F64L TTR and free λ of 3 mg/dL with 10% λ-restricted plasma cells; and a 70-year-old woman with GI involvement (TTR-positive by IHC) with hereditary variant T60A TTR and free λ of 2700 mg/dL with 30% λ-restricted plasma cells. All 3 patients had abnormal κ:λ ratios.
AA indicates secondary amyloid due to serum amyloid A protein; AL, light-chain amyloid; ATTR, familial transthyretin amyloidosis; MG, monoclonal gammopathy; and TTR, transthyretin.