Table I.
Baseline clinical features of 91 patients with systemic anaplastic large cell lymphoma.
| Clinical features at diagnosis | ALCL by ALK status, n (%) | ALK-negative ALCL by subtype n (%) | All ALCLn (%) | |||
|---|---|---|---|---|---|---|
| ALK-positive | ALK-negative | DUSP22 | P63 | Triple negative | ||
| Total n | 29 | 62 | 12 | 1 | 49 | 91 |
| Median age, years | 33 | 62 | 61–5 | 79 | 62 | 57 |
| (range) | 7–79 | 9–96 | 27–86 | 9–96 | 7–96 | |
| Male | 17 (59) | 46 (74) | 10 (83) | 0 | 36 (72) | 63 (69) |
| B symptoms | 19 (66) | 30 (48) | 5 (42) | 1 (100) | 24 (49) | 48 (53) |
| Bulky (≥10 cm) | 6 (21) | 8 (13) | 0 | 0 | 8 (17) | 14 (15) |
| PS ≥ 2 | 12 (44) | 24 (55) | 3 (25) | 1 (100) | 15 (37–5) | 30 (33) |
| Stage | ||||||
| 1 | 4 (14) | 10 (16) | 2 (12) | 0 | 8 (16) | 14 (15) |
| 2 | 7 (24) | 13 (21) | 1 (8) | 0 | 12 (24–5) | 20 (22) |
| 3 | 5 (17) | 11 (18) | 2 (12) | 0 | 9 (18) | 16 (18) |
| 4 | 13 (45) | 45 (28) | 7 (58) | 1 (100) | 20 (41) | 41 (45) |
| Extranodal (any) | 21 (72) | 39 (63) | 8 (67) | 1 (100) | 30 (61) | 60 (66) |
| Skin | 3 (10) | 9 (15) | 3 (25) | 0 | 6 (12) | 12 (13) |
| Soft tissue | 8 (28) | 13 (21) | 0 | 0 | 13 (27) | 21 (23) |
| Bone | 0 | 11 (18) | 4 (33)* | 0 | 7 (14) | 11 (12) |
| GI Tract | 1 (3) | 3 (5) | 3 (6) | 0 | 3 (6) | 4 (4) |
| Liver | 1 (3) | 4 (7) | 1 (8) | 0 | 3 (6) | 5 (6) |
| Lung | 4 (14) | 4 (7) | 1 (8) | 0 | 5 (10) | 10 (11) |
| Pleural effusion | 3 (10) | 6 (10) | 1 (8) | 1 (100) | 3 (6) | 7 (8) |
| Bone marrow/peripheral blood | 4 (14) | 8 (13) | 2 (12)* | 1 (100) | 5 (10) | 12 (13) |
| Extranodal >1 | 7 (24) | 21 (34) | 4 (33) | 1 (100) | 16 (33) | 28 (31) |
| LDH elevated | 10 (40) | 25 (4) | 6 (50) | 0 | 19 (42) | 48 (53) |
| LDH >2× ULN | 3 (10) | 12 (19) | 5 (42) | 0 | 12 (24) | 15 (17) |
| IPI | ||||||
| 0–1 | 8 (32) | 19 (31) | 5 (42) | 0 | 14 (31) | 27 (30) |
| 2 | 10 (40) | 11 (18) | 2 (12) | 0 | 9 (20) | 21 (23) |
| 3 | 3 (12) | 12 (20) | 2 (12) | 0 | 10 (22) | 15 (17) |
| 4–5 | 4 (16) | 15 (25) | 3 (25) | 1 (100) | 12 (27) | 20 (22) |
| Primary therapy | ||||||
| CHOP/CHOP(like) | 24 (83) | 46 (74) | 11 (92) | 0 | 35 (72) | 71 (78) |
| RT or surgery alone | 0 | 6 (10) | 1 (8) | 0 | 5 (10) | 6 (8) |
| Other | 3 (10) | 4 (7) | 0 | 0 | 4 (8) | 7 (8) |
| None | 2 (7) | 5 (9) | 0 | 1 (100) | 5 (10) | 7 (8) |
| Consolidative ASCT | - | - | 1 | - | - | - |
Missing data: LDH missing (ALK-positive n = 4; triple negative n = 4); IPI missing n = 8 (ALK-positive n = 4; triple negative n = 4); Mass size (n = 2); PS n = 12.
ALCL, anaplastic large cell lymphoma; ALK, anaplastic lymphoma kinase; ASCT, autologous stem cell transplantation; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; GI, gastrointestinal; IPI, International Prognostic Index; LDH, lactate dehydrogenase; PS, performance status; RT, radiotherapy; ULN, upper limit of normal.
*
One patient had bone and bone marrow involvement. Estimates were rounded.