1. T cell lymphomas- nodal, cutaneous, leukemic. T cell lymphomas comprise 10-15% of mature lymphomas from post thymic T cells.
2. Nodal approximately 60%. 3 main groups- PTCL NOS, Anaplastic (ALK positive and ALK neg), T follicular helper cell nodal ( includes angioimmunoblastic).
3. Standard of care for CD 30+ T cell lymphoma is BV+ CHP based on ECHELON 2 which showed OS and PFS benefit over previous SOC which was CHOP.
4. 5 yr OS for all PTCL with CHOP= 30%, ALK+ anaplastic is the best with 5 yr OS 70%. This group also did the best with CHOEP and BV- CHEP. PFS 48 months versus 20 months BV- CHP versus CHOP in ECHELON 2.
5. Consolidation in CR1 is standard with auto SCT and in first relapse after salvage with allo- SCT. Anaplastic ALK+ low risk do not need auto SCT in CR1.
6. ALK neg anaplastic but with DUSP22 mutation acts like ALK+ anaplastic.
7. GATA 3 mutation is a bad prognostic indicator
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