Demographic and clinical characteristics of patients at baseline
| Characteristic . | TN (n = 24) . | R/R (n = 53) . | Total (N = 77) . |
|---|---|---|---|
| Age | |||
| Years, median (range) | 65 (40-87) | 68 (45-87) | 67 (40-87) |
| >75 y, n (%) | 3 (12.5) | 13 (24.5) | 16 (20.8) |
| Male, n (%) | 16 (67) | 45 (85) | 61 (79) |
| ECOG performance status score, n (%) | |||
| 0/1 | 24 (100) | 50 (94) | 74 (96) |
| 2 | 0 (0) | 3 (6) | 3 (4) |
| Serum IgM (g/L)* | |||
| Median (range) | 43.9 (5.3-91.9) | 29.4 (1.2-88.5) | 32.4 (1.2-91.9) |
| ≥40 g/L, n (%) | 13 (54) | 11 (21) | 24 (31) |
| Baseline hemoglobin (g/L) | |||
| Median (range) | 100.5 (68-132) | 106.0 (63-155) | 105.0 (63-155) |
| ≤110 g/L, n (%) | 14 (58) | 32 (60) | 46 (60) |
| Extramedullary disease, n (%) | |||
| Lymphadenopathy† | 13 (54) | 26 (49) | 39 (51) |
| Splenomegaly | 9 (38) | 17 (32) | 26 (34) |
| Bone marrow infiltration, median (range) | |||
| Cellularity | 42.5 (10-95) | 27.5 (0-94) | 35 (0-95) |
| No. of prior systemic therapies | NA | 2 (1-8) | 2 (1-8) |
| Genotype, n (%)‡ | |||
| MYD88L265P/CXCR4WT | 14 (58.3) | 26 (49.1) | 40 (51.9) |
| MYD88L265P/CXCR4WHIM | 4 (16.7) | 7 (13.2) | 11 (14.3) |
| MYD88L265P/CXCR4FS | 2 (8.3) | 4 (7.5) | 6 (7.8) |
| MYD88L265P/CXCR4NS | 2 (8.3) | 3 (5.7) | 5 (6.5) |
| MYD88L265P/CXCR4UNK | 2 (8.3) | 5 (9.4) | 7 (9.1) |
| MYD88WT/CXCR4WT | 3 (12.5) | 8 (15.1) | 11 (14.3) |
| Characteristic . | TN (n = 24) . | R/R (n = 53) . | Total (N = 77) . |
|---|---|---|---|
| Age | |||
| Years, median (range) | 65 (40-87) | 68 (45-87) | 67 (40-87) |
| >75 y, n (%) | 3 (12.5) | 13 (24.5) | 16 (20.8) |
| Male, n (%) | 16 (67) | 45 (85) | 61 (79) |
| ECOG performance status score, n (%) | |||
| 0/1 | 24 (100) | 50 (94) | 74 (96) |
| 2 | 0 (0) | 3 (6) | 3 (4) |
| Serum IgM (g/L)* | |||
| Median (range) | 43.9 (5.3-91.9) | 29.4 (1.2-88.5) | 32.4 (1.2-91.9) |
| ≥40 g/L, n (%) | 13 (54) | 11 (21) | 24 (31) |
| Baseline hemoglobin (g/L) | |||
| Median (range) | 100.5 (68-132) | 106.0 (63-155) | 105.0 (63-155) |
| ≤110 g/L, n (%) | 14 (58) | 32 (60) | 46 (60) |
| Extramedullary disease, n (%) | |||
| Lymphadenopathy† | 13 (54) | 26 (49) | 39 (51) |
| Splenomegaly | 9 (38) | 17 (32) | 26 (34) |
| Bone marrow infiltration, median (range) | |||
| Cellularity | 42.5 (10-95) | 27.5 (0-94) | 35 (0-95) |
| No. of prior systemic therapies | NA | 2 (1-8) | 2 (1-8) |
| Genotype, n (%)‡ | |||
| MYD88L265P/CXCR4WT | 14 (58.3) | 26 (49.1) | 40 (51.9) |
| MYD88L265P/CXCR4WHIM | 4 (16.7) | 7 (13.2) | 11 (14.3) |
| MYD88L265P/CXCR4FS | 2 (8.3) | 4 (7.5) | 6 (7.8) |
| MYD88L265P/CXCR4NS | 2 (8.3) | 3 (5.7) | 5 (6.5) |
| MYD88L265P/CXCR4UNK | 2 (8.3) | 5 (9.4) | 7 (9.1) |
| MYD88WT/CXCR4WT | 3 (12.5) | 8 (15.1) | 11 (14.3) |
ECOG, Eastern Cooperative Oncology Group; FS, frameshift mutation; NA, not applicable; NS, nonsense mutation; UNK, unknown.
On the basis of nephelometric assessment (n = 74) or in the absence of a quantitative IgM level with SPEP.
Thirty-one patients had baseline lymphadenopathy on the basis of CT imaging alone.
Genotype data were obtained from baseline bone marrow aspirate samples, or if not available, postbaseline samples. Eight patients (1 TN and 7 R/R) did not provide bone marrow samples for MYD88/CXCR4 genomic profiling. Five of 8 patients had enrolled prior to protocol requirement for bone marrow analysis for MYD88/CXCR4, and 3 of 8 patients did not sign the optional informed consent for genetic testing of bone marrow.