Clinical and cytogenetic data of our cases and prior outcomes of ALK-positive LBCL
| Case . | Age at diagnosis . | Therapy . | ALK staining . | Morphology . | Immunophenotype . | LDH at diagnosis (U/L); reference range: 120-246 U/L . | EBV (via EBER) . | Response . | Time since diagnosis . |
|---|---|---|---|---|---|---|---|---|---|
| Case 1 | 23 | CHOP × 3 Radiation Crizotinib | Cytoplasmic and golgi | Neoplastic cells with plasmablastic features and cytoplasmic ALK protein | ALK+, CD45 (wk), CD79a (wk), CD138 (subset), EMA+, MUM1+ CD3-, CD20-, CD30- CD43-, HHV8-, Melan-A-, OCT4-, Pan-keratin-, S100- | 209 | Negative | Complete response for 6 y | 6 y |
| Case 2 | 46 | CHOP × 1 Radiation Crizotinib Alectinib Lorlatinib | Granular cytoplasmic | Diffuse sheets of large, very abnormal appearing lymphoid cells with abundant cytoplasm, pale nuclei, and prominent central nucleoli | ALK+, CD45+, CD138+, PAX5 (weak), CD4 (weak), CD30 (weak, subset), IgA heavy chain+, kappa light chain (weak) CD20-, CD3-, CD5-, CD8-, S100-, MCK-, lambda light chain- | 353 | Unknown | Complete response for 6 months | 1.8 y |
| Wass et al13 | 27 | CHOP × 6 HDC + ASCT Crizotinib | 233 mmol/L | Unknown | Brief response to crizotinib; PD within 21 d | ||||
| Li et al14 | 21 | Splenectomy CHOP × 5 ICE × 5 GemOx+Dex+ Crizotinib | Cytoplasmic granular | Large cell with plasmacytic differentiation | CD138+, CD4+, MUM1+, ALK+, CD30 (weak), CD38 (weak), CD43 (weak), CD57 (weak) CD20-, CD15-, CD79a-, CD3-, CD7-, S100- | 858 | Negative | Progressive disease | Death within 2 y of diagnosis |
| Mehra et al15 | 23 | ALCL 99 protocol BV v 2 GemOx × 1 Crizotinib × 2 weeks | Cytoplasmic and paranuclear | CD30+, ALK+, CD45+, EMA+, BCL-6+, CD10+, CD15+ | 1000 | Unknown | Progressive disease | Death within 4 mo of diagnosis | |
| Soumerai et al17 | 25-50 (4 patients) | Chemotherapy (CHOP, EPOCH, DHAP, ICE, lenalidomide, bendamustine, Gem-Ox, Elotuzumab), crizotinib | Large lymphoid cells with plasmablastic morphology | CD20-, CD19-, PAX5-, CD3-, CD2-, CD5- | Elevated in 3 of 4 patients | Unknown | 2 patients with complete response and 2 patients with PD |
| Case . | Age at diagnosis . | Therapy . | ALK staining . | Morphology . | Immunophenotype . | LDH at diagnosis (U/L); reference range: 120-246 U/L . | EBV (via EBER) . | Response . | Time since diagnosis . |
|---|---|---|---|---|---|---|---|---|---|
| Case 1 | 23 | CHOP × 3 Radiation Crizotinib | Cytoplasmic and golgi | Neoplastic cells with plasmablastic features and cytoplasmic ALK protein | ALK+, CD45 (wk), CD79a (wk), CD138 (subset), EMA+, MUM1+ CD3-, CD20-, CD30- CD43-, HHV8-, Melan-A-, OCT4-, Pan-keratin-, S100- | 209 | Negative | Complete response for 6 y | 6 y |
| Case 2 | 46 | CHOP × 1 Radiation Crizotinib Alectinib Lorlatinib | Granular cytoplasmic | Diffuse sheets of large, very abnormal appearing lymphoid cells with abundant cytoplasm, pale nuclei, and prominent central nucleoli | ALK+, CD45+, CD138+, PAX5 (weak), CD4 (weak), CD30 (weak, subset), IgA heavy chain+, kappa light chain (weak) CD20-, CD3-, CD5-, CD8-, S100-, MCK-, lambda light chain- | 353 | Unknown | Complete response for 6 months | 1.8 y |
| Wass et al13 | 27 | CHOP × 6 HDC + ASCT Crizotinib | 233 mmol/L | Unknown | Brief response to crizotinib; PD within 21 d | ||||
| Li et al14 | 21 | Splenectomy CHOP × 5 ICE × 5 GemOx+Dex+ Crizotinib | Cytoplasmic granular | Large cell with plasmacytic differentiation | CD138+, CD4+, MUM1+, ALK+, CD30 (weak), CD38 (weak), CD43 (weak), CD57 (weak) CD20-, CD15-, CD79a-, CD3-, CD7-, S100- | 858 | Negative | Progressive disease | Death within 2 y of diagnosis |
| Mehra et al15 | 23 | ALCL 99 protocol BV v 2 GemOx × 1 Crizotinib × 2 weeks | Cytoplasmic and paranuclear | CD30+, ALK+, CD45+, EMA+, BCL-6+, CD10+, CD15+ | 1000 | Unknown | Progressive disease | Death within 4 mo of diagnosis | |
| Soumerai et al17 | 25-50 (4 patients) | Chemotherapy (CHOP, EPOCH, DHAP, ICE, lenalidomide, bendamustine, Gem-Ox, Elotuzumab), crizotinib | Large lymphoid cells with plasmablastic morphology | CD20-, CD19-, PAX5-, CD3-, CD2-, CD5- | Elevated in 3 of 4 patients | Unknown | 2 patients with complete response and 2 patients with PD |