| Criterion | Mayo series (n = 99) | Soubrier, et al. (n = 25) | Nakanishi, et al. (n = 102) |
| Polyneuropathy | 100 | 100 | 100 |
| Organomegaly | 50 | NA | NA |
| Hepatomegaly | 24 | 68 | 78 |
| Splenomegaly | 22 | 52 | 35 |
| Lymphadenopathy | 26 | 52 | 61 |
| Endocrinopathy | 67 | NA | NA |
| Diabetes mellitus | 3 | 36 | 25 |
| Monoclonal plasma cell disorder | 100 | 100 | 75 |
| Skin changes | 68 | NA | NA |
| Hyperpigmentation | 46 | 48 | 93 |
| Hypertrichosis | 26 | 24 | 74 |
