Localised amyloid disease
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16 |
SECTION 2B - Diseases with monoclonal light chain deposition |
| Localised amyloid disease |
Rather than being a systemic disease, amyloid deposition may be limited to single organs. The distribution depends upon the biochemical nature of the amyloid fibril protein and as in AL amyloidosis, light chain fragments may be involved. Serum FLCs have been evaluated in a variety of such patients attending the UK National Amyloidosis Centre and are shown in Table 16.1. An enlarged series of 235 cases was reported by Goodman et al [1].
Overall, elevated serum FLCs are less commonly observed than in systemic AL amyloidosis and even when present, the concentrations are lower. Serum FLC concentrations may, therefore, assist in distinguishing the different types of amyloid disease and systemic from localised light chain amyloid disease.
Site of amyloid deposits |
Number of patients | Monoclonal proteins* | Abnormal κ/λ ratios |
|---|---|---|---|
| Bone | 7 | 3 (43%) | 6 (88%) |
| Bladder | 25 | 1 (4%) | 3 (12%) |
| Bowel | 10 | 4 (40%) | 3 (30%) |
| Bronchial | 13 | 2 (15%) | 1 (8.0%) |
| Nodular pulmonary | 13 | 3 (23%) | 3 (23%) |
| Laryngeal | 22 | 0 | 1 (4.5%) |
| Nasopharynx | 16 | 1 (6%) | 2 (13%) |
| Skin | 18 | 2 (11%) | 2 (11%) |
| Occular | 10 | 2 (20%) | 2 (20%) |
| Lymph Node | 16 | 3 (19%) | 5 (31%) |
| Miscellaneous | 6 | 0 | 1 (17%) |
Table 16.1. Frequency of monoclonal proteins in patients with localised amyloid disease. *Serum monoclonal proteins or light chain proteinuria identified by electrophoretic tests. (Courtesy of PN Hawkins).
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