Table 1.
Pathological and Clinical Features of 7 Cases of High-Grade B-Cell Lymphoma With Triple or Quadruple Hits Involving CCND1 Gene Rearrangement.
| Case | Age/Gender | Clinical History | Diagnosis | Stage | FISH/Cytogenetics | Immunohistochemistry | Treatment | Outcome | Ref |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 37/M | Diffuse lymphadenopathy, breast mass, Bell’s palsy | High-grade B-cell lymphoma | IV-B | CCND1 RMYC RBCL2 R | Cyclin D1+ CD5- SOX11- CD10+ BCL2+ BCL6- MUM1+ Ki67 90% TDT focal + | EPOCH-R, HSCT | DOD (18 mo) | |
| 2 | 74/F | Mediastinal mass | High-grade B-cell lymphoma | II | CCND1 RMYC RBCL2 RBCL6 R | Cyclin D1+ CD5- SOX11-CD10- BCL2+ BCL6+ MUM1+ Ki67 90% | R-CHOP | DOD (8 mo) | |
| 3 | 51/F | Diffuse lymphadenopathy | DLBCL | III | CCND1 RMYC RBC2 RBCL6 R | CD10+ BCL2+ BCL6+ MUM1+ Ki67 80% | R-CHOP | A, NED (30 mo) | 10 |
| 4 | 81/F | Bone marrow involved by B-cell lymphoma | DLBCL | Unknown | BCL6 R/t(3;18)(q27;q21) MYC R/t(8;14)(q24;q32) CCND1 R/t(11;14)(q13;q32) BCL2 R/t(14;18)(q32;q21) | Unknown | Cyclophosphamide containing chemotherapy | Death due to unknown causes (9 d) | 11 |
| 5 | 68/M | Bone marrow involved by B-cell lymphoma | BL/B-ALL | Unknown | BCL6 R/t(2;3)(p12;q27) MYC R/t(8;14)(q24;q32) CCND1 R/t(11;14)(q13;q32) BCL2 R/t(14;18)(q32;q21) | Unknown | Unknown | DOD (6 d) | 11 |
| 6 | 79/M | Paranesthesia for 4 mo and diffuse lymphadenopathy | DLBCL | III/IV | CCND1 RMYC RBCL2 RBCL6 R | Cyclin D1+ CD5- SOX11- CD10- BCL2+ BCL6+Ki67 80% | RICE | DOD (4 mo) | 12 |
| 7 | Unknown | Meningeal lymphomatosis and diffuse lymphadenopathy | B-cell lymphoma | Unknown | t(3;14)(q27;q32) t(8;14)(q24;q32) t(11;22)(q13;q11) t(14;18)(q32;q21) | Cyclin D1+ CD5- CD10+ BCL2+ BCL6+ TDT-Ki67 60% | Doxorubicin containing chemo/rituximab | DOD | 13 |
ALL = acute lymphoblastic leukemia, BL = Burkitt Lymphoma, CR = complete response, DLBCL = diffuse large B-cell lymphoma, EPOCH-R = rituximab, cyclophosphamide, hydroxydaunomycin, prednisone, etoposide, FISH = fluorescence in situ hybridization, HSCT = hematopoietic stem cell transplant, LAD = lymphadenopathy, R = gene rearranged by FISH, R-CHOP = rituximab, cyclophosphamide, hydroxydaunomycin, vincristine sulfate, prednisone, RICE = rituximab, ifosfamide, carboplatin, etoposide.