Overview of clinical characteristics, treatment, follow-up, and results of 25 patients with IVLBCL
| Characteristic . | n/N (%) . |
|---|---|
| Sex | |
| Men | 9/25 (36) |
| Women | 16/25 (64) |
| Age at diagnosis, y | |
| Median | 64 |
| Range | 49-85 |
| Presence of B symptoms | 18/22 (82) |
| Disease localization | |
| Skin | 12/25 (48) |
| Bone marrow | 8/24 (33) |
| Brain | 7/24 (29) |
| Lung | 7/24 (29) |
| Spleen | 5/24 (21) |
| Liver | 4/24 (17) |
| Lymph nodes | 4/24 (17) |
| Kidney | 3/24 (13) |
| Other (thyroid, testis, [cardiac] muscle, soft tissue) | 5/24 (21) |
| Skin-limited disease | 6/24 (25) |
| Ann Arbor stage* | |
| IE (skin, brain, kidney) | 9/24 (38) |
| IV | 15/24 (63) |
| First-line therapy† | |
| Immunochemotherapy‡ | 6/22 (27) |
| Chemotherapy§ | 6/22 (27) |
| Immunotherapy (rituximab only) | 1/22 (5) |
| Radiotherapy | 3/22 (14) |
| Supportive care with/without steroids | 5/22 (23) |
| Surgery + watchful waiting (subcutaneous lipoma) | 1/22 (5) |
| Second-line therapy | |
| Immunochemotherapy‡ | 2/22 (9) |
| Chemotherapy§ | 1/22 (5) |
| Radiotherapy | 2/22 (9) |
| Survival status | |
| Died, disease related | 14/25 (56) |
| Died, disease unrelated | 5/25 (20) |
| Alive | 6/25 (24) |
| Median overall survival time, years‖ | 1.68 |
| Immunohistochemistry | |
| CD20 | 25/25 (100) |
| CD10 | 2/25 (8) |
| BCL6 | 14/24 (58) |
| MUM1 | 18/24 (75) |
| BCL2 | 23/24 (96) |
| IgM | 21/23 (91) |
| MYC | 15/22 (68) |
| CD5 | 13/25 (52) |
| Cyclin D1 | 0/25 (0) |
| CD30 | 0/24 (0) |
| EBER ISH | 0/24 (0) |
| MYC FISH | 0/15 (0) |
| Targeted next-generation sequencing | |
| MYD88 (exon 3/exon 5) | 11/25 (44) |
| CD79B (exon 5/exon 6) | 6/23 (26) |
| CD79A (exon 5) | 0/24 (0) |
| CARD11 (exon 6) | 0/24 (0) |
| EZH2 (exon 16) | 1/25 (4) |
| Characteristic . | n/N (%) . |
|---|---|
| Sex | |
| Men | 9/25 (36) |
| Women | 16/25 (64) |
| Age at diagnosis, y | |
| Median | 64 |
| Range | 49-85 |
| Presence of B symptoms | 18/22 (82) |
| Disease localization | |
| Skin | 12/25 (48) |
| Bone marrow | 8/24 (33) |
| Brain | 7/24 (29) |
| Lung | 7/24 (29) |
| Spleen | 5/24 (21) |
| Liver | 4/24 (17) |
| Lymph nodes | 4/24 (17) |
| Kidney | 3/24 (13) |
| Other (thyroid, testis, [cardiac] muscle, soft tissue) | 5/24 (21) |
| Skin-limited disease | 6/24 (25) |
| Ann Arbor stage* | |
| IE (skin, brain, kidney) | 9/24 (38) |
| IV | 15/24 (63) |
| First-line therapy† | |
| Immunochemotherapy‡ | 6/22 (27) |
| Chemotherapy§ | 6/22 (27) |
| Immunotherapy (rituximab only) | 1/22 (5) |
| Radiotherapy | 3/22 (14) |
| Supportive care with/without steroids | 5/22 (23) |
| Surgery + watchful waiting (subcutaneous lipoma) | 1/22 (5) |
| Second-line therapy | |
| Immunochemotherapy‡ | 2/22 (9) |
| Chemotherapy§ | 1/22 (5) |
| Radiotherapy | 2/22 (9) |
| Survival status | |
| Died, disease related | 14/25 (56) |
| Died, disease unrelated | 5/25 (20) |
| Alive | 6/25 (24) |
| Median overall survival time, years‖ | 1.68 |
| Immunohistochemistry | |
| CD20 | 25/25 (100) |
| CD10 | 2/25 (8) |
| BCL6 | 14/24 (58) |
| MUM1 | 18/24 (75) |
| BCL2 | 23/24 (96) |
| IgM | 21/23 (91) |
| MYC | 15/22 (68) |
| CD5 | 13/25 (52) |
| Cyclin D1 | 0/25 (0) |
| CD30 | 0/24 (0) |
| EBER ISH | 0/24 (0) |
| MYC FISH | 0/15 (0) |
| Targeted next-generation sequencing | |
| MYD88 (exon 3/exon 5) | 11/25 (44) |
| CD79B (exon 5/exon 6) | 6/23 (26) |
| CD79A (exon 5) | 0/24 (0) |
| CARD11 (exon 6) | 0/24 (0) |
| EZH2 (exon 16) | 1/25 (4) |
EBER-ISH, Epstein-Barr virus early RNA in situ hybridization; IgM, immunoglobulin M; MYC FISH, fluorescence in situ hybridization with breakapart probes for MYC.
Staging procedures were not performed in 1 patient with skin involvement.
Three patients were diagnosed at autopsy.
Immunochemotherapy consisted in first-line of R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone; n = 4), R-CHOP with methotrexate (n = 1), and R-CVP (rituximab plus cyclophosphamide, vincristine, and prednisone; n = 1) and in second-line of R-CHOP (n = 1) and R-DHAP (rituximab plus dexamethasone, cytarabine, and cisplatin; n = 1).
Chemotherapy consisted in first-line of CHOP or CHOP-like regimens (n = 4), MBVP (methotrexate, carmustine, teniposide, and prednisone; n = 1), and vincristine with steroids (n = 1) and in second-line of IMVP (ifosfamide, methotrexate, etoposide, and prednisone) and DHAP followed by autologous stem-cell transplantation (n = 1).
Patients diagnosed at autopsy were excluded.