TABLE 1.
Key baseline characteristics
|
Cohort A Treatment naïve (N = 18) |
Cohort B Relapsed/refractory (N = 9) |
All (N = 27) | |
|---|---|---|---|
| Age | |||
| Median (years) | 70.5 (50–82) | 71 (60–83) | 71 (50–83) |
| <65 years | 6 (33) | 3 (33) | 9 (33) |
| 65–74 years | 5 (28) | 4 (44) | 9 (33) |
| ≥75 years | 7 (39) | 2 (22) | 9 (33) |
| Serum IgM | |||
| Median (mg/dl) | 3787.5 (1392–6340) | 2105 (730–6930) | 3600 (730–6930) |
| >4000 mg/dl | 7 (39) | 2 (22) | 9 (33) |
| Hemoglobin | |||
| Median (g/dl) | 10.45 (8.0–15.3) | 12.2 (9.1–13.9) | 10.6 (8.0–15.3) |
| ≤10 g/dl | 7 (39) | 4 (44) | 11 (41) |
| Symptoms observed in ≥10% patients | |||
| Recurrent fever, night sweats, weight loss, or fatigue | 5 (28) | 1 (11) | 6 (22) |
| Hyperviscosity | 6 (33) | 1 (11) | 7 (26) |
| Peripheral neuropathy due to WM | 2 (11) | 1 (11) | 3 (11) |
| Genotype a | |||
| MYD88 wildtype/CXCR4 WHIM | 1 (6) | 0 | 1 (4) |
| MYD88 L265P/CXCR4 wildtype | 13 (76) | 9 (100) | 22 (85) |
| MYD88 L265P/CXCR4 WHIM | 3 (18) | 0 | 3 (12) |
Data are numbers of patients (%) or median (range).
a
Gene mutation data were missing in one patient in Cohort A.