Main clinical features of the 31 patients at the time of diagnosis
| . | N (%) . | Median (range) . |
|---|---|---|
| Male | 19 (61.3) | NA |
| Age at diagnosis, y | NA | 44 (32-68) |
| Time from symptoms to diagnosis, mo | NA | 19 (4-80) |
| Polyneuropathy | 31 (100) | NA |
| Motor | 31 (100) | NA |
| Sensor | 29 (93.5) | NA |
| CSF protein, g/L | NA | 1.32 (0.43-3.12) |
| ONLS score | ||
| Upper limb | NA | 1 (0-4) |
| Lower limb | NA | 3 (1-6) |
| Overall | NA | 4 (1-10) |
| Organomegaly | 28 (90.3) | NA |
| Lymphadenopathy | 20 (80.6) | NA |
| Castleman disease* | 5/11 (45.6) | NA |
| Splenomegaly | 21 (67.7) | NA |
| Hepatomegaly | 13 (41.9) | NA |
| Endocrinopathy | 27 (87.1) | NA |
| Diabetes mellitus | 5 (16.1) | NA |
| Hypothyroidism | 21 (67.7) | NA |
| Increased ACTH value | 10 (32.2) | NA |
| Gonadal axis abnormality | 20 (64.4) | NA |
| M protein | 31 (100) | NA |
| IgA-λ | 23 (74.2) | NA |
| IgG-λ | 8 (25.8) | NA |
| Skin change | 29 (93.5) | NA |
| Hyperpigmentation | 29 (93.5) | NA |
| Hemangioma | 16 (51.6) | NA |
| Hypertrichosis | 13 (41.9) | NA |
| Thickening | 11 (35.5) | NA |
| Bone lesion | ||
| Sclerotic | 4 (13.1) | NA |
| Lytic | 4 (13.1) | NA |
| Mixed | 0 (0) | |
| Extravascular overload | 29 (93.5) | NA |
| Edema | 26 (83.4) | NA |
| Ascites | 15 (48.4) | NA |
| Pleural effusion | 12 (38.7) | NA |
| Pericardial effusion | 18 (58.1) | NA |
| Pulmonary hypertension, mmHg | 15 (48.4) | 49 (39-100) |
| Papilledema | 19 (61.3) | NA |
| Thrombocytosis | 18 (58.1) | NA |
| 24-h urine protein, > 0.5 g | 7 (22.6) | NA |
| CCrl, < 60 mL/min | 9 (29) | NA |
| . | N (%) . | Median (range) . |
|---|---|---|
| Male | 19 (61.3) | NA |
| Age at diagnosis, y | NA | 44 (32-68) |
| Time from symptoms to diagnosis, mo | NA | 19 (4-80) |
| Polyneuropathy | 31 (100) | NA |
| Motor | 31 (100) | NA |
| Sensor | 29 (93.5) | NA |
| CSF protein, g/L | NA | 1.32 (0.43-3.12) |
| ONLS score | ||
| Upper limb | NA | 1 (0-4) |
| Lower limb | NA | 3 (1-6) |
| Overall | NA | 4 (1-10) |
| Organomegaly | 28 (90.3) | NA |
| Lymphadenopathy | 20 (80.6) | NA |
| Castleman disease* | 5/11 (45.6) | NA |
| Splenomegaly | 21 (67.7) | NA |
| Hepatomegaly | 13 (41.9) | NA |
| Endocrinopathy | 27 (87.1) | NA |
| Diabetes mellitus | 5 (16.1) | NA |
| Hypothyroidism | 21 (67.7) | NA |
| Increased ACTH value | 10 (32.2) | NA |
| Gonadal axis abnormality | 20 (64.4) | NA |
| M protein | 31 (100) | NA |
| IgA-λ | 23 (74.2) | NA |
| IgG-λ | 8 (25.8) | NA |
| Skin change | 29 (93.5) | NA |
| Hyperpigmentation | 29 (93.5) | NA |
| Hemangioma | 16 (51.6) | NA |
| Hypertrichosis | 13 (41.9) | NA |
| Thickening | 11 (35.5) | NA |
| Bone lesion | ||
| Sclerotic | 4 (13.1) | NA |
| Lytic | 4 (13.1) | NA |
| Mixed | 0 (0) | |
| Extravascular overload | 29 (93.5) | NA |
| Edema | 26 (83.4) | NA |
| Ascites | 15 (48.4) | NA |
| Pleural effusion | 12 (38.7) | NA |
| Pericardial effusion | 18 (58.1) | NA |
| Pulmonary hypertension, mmHg | 15 (48.4) | 49 (39-100) |
| Papilledema | 19 (61.3) | NA |
| Thrombocytosis | 18 (58.1) | NA |
| 24-h urine protein, > 0.5 g | 7 (22.6) | NA |
| CCrl, < 60 mL/min | 9 (29) | NA |
NA indicates not applicable; CSF, cerebrospinal fluid; ACTH, adrenocorticotropic hormone; and CCrl, creatinine clearance rate.
Five of 11 patients who underwent lymph node biopsy were diagnosed with Castleman disease.