Table 2.
Main characteristics of nodal peripheral T-cell lymphomas.
| PTCL-NOS | AITL | ALCL-ALK+ | ALCL-ALK- | |
|---|---|---|---|---|
| Age Gender predominance |
>60 years Male |
>60 years Male |
<40 years Male |
>60 years Male |
| Clinical-laboratory markers and prognosis | Frequent extranodal involvement; Very aggressive course |
Nodal predominance; Splenomegaly; Autoimmune disorders; Cutaneous rash; Eosinophilia; Hypergammaglobulinemia |
Aggressive course; Low and low-intermediate IPI; Good prognosis, particularly if low IPI |
Aggressive course, but better than PTCL-NOS; Intermediate prognosis between ALCL-ALK+ and PTCL-NOS |
| Histopathology | Monomorphic infiltrate by small and medium lymphoid cells | Polymorphic infiltrate; Vascular proliferation; Follicular dendritic cell proliferation; Large immunoblasts EBV+ |
Hallmark cells; Mimic metastatic carcinoma |
Hallmark cells; Mimic metastatic carcinoma |
| Immunohistochemistry | CD3+ (75%), CD4+, CD7−, CD30+ or− | CD10+, BCL6+, CXCL13+, PD1+, ICOS+, EBV+ | CD3+ (25%), CD4+, CD30+ high, ALK1+ | CD3+ (25%), CD4+, CD30+ high, ALK– |
| Cytogenetics | t(5;9) ITK-SYK | +3, +5, +X | t(2;5) NPM-ALK | Non-specific |
| 5-year overall survival with CHOP | 19% | 18% | 70–80% | 49% |
| Treatment option | 6–8 CHO(E)P + aHSCT | 6–8 (R)CHO(E)P + aHSCT | 6–8 CHO(E)P | 6–8 CHO(E)P + aHSCT |
| Potential new therapeutic agents | Pralatrexate Vorinostat |
Bevacizumab Lenalidomide |
Crizotinib Brentuximab vedotin |
Brentuximab vedotin |
PTCL-NOS: peripheral T-cell lymphoma not otherwise specified; AITL: angioimmunoblastic T-cell lymphoma; ALCL-ALK+: anaplastic large cell lymphoma-anaplastic lymphoma kinase positive; ALCL-ALK−: anaplastic large cell lymphoma-anaplastic lymphoma kinase negative; IPI: International Prognostic Index; EBV: Epstein–Barr virus; CHO(E)P: cyclophosphamide, doxorubicin, vincristine, prednisolone; aHSCT: autologous hematopoietic stem cell transplantation.