Table 1.
Baseline | N (%) | Median (range) |
---|---|---|
Male | 28 (68) | NA |
Age at diagnosis, years | NA | 49 (21–70) |
Time from symptoms to diagnose, months | NA | 7 (1-84) |
Polyneuropathy | 41 (100) | NA |
ONLS score | ||
Upper limb | NA | 1 (0–4) |
Lower limb | NA | 2 (1–7) |
Overall | NA | 4 (1–10) |
Organomegaly | 41 (100) | NA |
Lymphadenopathy | 38 (93) | NA |
Splenomegaly | 22 (54) | NA |
Hepatomegaly | 12 (29) | NA |
Endocrinopathy | 41 (100.0) | NA |
Diabetes mellitus | 6 (15) | NA |
Hypothyroidism | 17 (42) | NA |
Gynecomastia (Male) | 21/28 (75) | NA |
Increased ACTH | 28/39 (72) | NA |
M-protein | 41 (100.0) | NA |
IgAλ | 25 (61) | NA |
IgAλ + IgGκ | 1 (2) | NA |
IgAλ + IgMλ | 1 (2) | NA |
IgGλ | 13 (32) | NA |
λ | 1(2) | NA |
Skin change | 41 (100.0) | NA |
Hyperpigmentation | 38 (93) | NA |
Hemangiomas | 33 (81) | NA |
White nails | 25 (61) | NA |
Hypertrichosis | 15 (37) | NA |
Acrocyanosis | 6 (15) | NA |
Dry skin and ichthyosis-like lesions | 9 (22) | NA |
Bone lesion | 31 (76) | NA |
Extravascular overload | 40 (98) | NA |
Edema | 39 (95) | NA |
Ascites | 17 (42) | NA |
Pleural effusion | 21 (51) | NA |
Periscardial effusion | 28 (68) | NA |
Serum VEGF, pg/ml | 40 (98) | 5155 (534–14328) |
Pulmonary hypertension, % | 3 (7) | 54 (51–93) |
Papilledema | 23 (56) | NA |
Erythrocytosis | 8 (20) | 138 (94–182) |
Thrombocytosis | 18 (44) | 332 (89–628) |
NA, not available; ONLS, overall neuropathy limitation scale; ACTH, adrenocorticotropic hormone; VEGF, vascular endothelial growth factor.