T Cell Lymphoma

Peripheral T cell lymphomas (PTCL) are aggressive mature T cell neoplasms comprising 10 to 15% of non-Hodgkin lymphomas.

• Subtypes: PTCL not otherwise specified (25%), angioimmunoblastic T cell lymphoma (AITL, TFH phenotype), anaplastic large cell lymphoma (ALCL; ALK positive favourable, ALK negative), NK/T cell lymphoma (EBV, nasal type), enteropathy associated T cell lymphoma, hepatosplenic T cell lymphoma, adult T cell leukaemia/lymphoma (HTLV-1), cutaneous T cell lymphomas.

• International Prognostic Index applies; 5 year survival 30 to 40% (ALK positive ALCL 70%).

• Frequent relapse after anthracycline chemotherapy.

• Generalised lymphadenopathy, B symptoms, hepatosplenomegaly, skin rash, pruritus, eosinophilia, polyclonal hypergammaglobulinaemia (AITL), autoimmune phenomena.

• Nasal obstruction and facial destruction in NK/T lymphoma; haemophagocytic syndrome.

• Bowel perforation in enteropathy associated lymphoma.

• Excisional biopsy with immunophenotype (CD3, CD4, CD8, CD30, ALK, CD5, CD2, TFH markers PD1, CXCL13, CD10, BCL6), T cell receptor clonality, EBER for NK/T.

• PET-CT, marrow biopsy, LDH, HTLV-1 serology, EBV DNA, HIV.

• Cell of origin and mutation profile (RHOA, TET2, DNMT3A, IDH2).

• Assess haemophagocytosis (ferritin, triglycerides).

• ALCL (ALK positive and CD30 positive): brentuximab vedotin 1.8 mg/kg with CHP (cyclophosphamide, doxorubicin, prednisolone) for 6 cycles.

• Other PTCL: CHOP or CHOEP (add etoposide 100 mg/m2 days 1 to 3 for younger patients) for 6 cycles; consolidation with autologous stem cell transplant in first remission for fit patients.

• NK/T lymphoma: localised, concurrent radiotherapy (50 Gy) with DeVIC or sequential asparaginase-based regimens (SMILE, P-GemOx); avoid anthracyclines.

• Relapse or refractory: pralatrexate 30 mg/m2 weekly for 6 of 7 weeks, romidepsin 14 mg/m2 days 1, 8, 15, belinostat 1000 mg/m2, brentuximab for CD30 positive, duvelisib, azacitidine (AITL), chidamide, allogeneic transplant.

• Adult T cell leukaemia/lymphoma: zidovudine with interferon alfa for leukaemic types, chemotherapy (VCAP-AMP-VECP) for acute types, mogamulizumab.

• Supportive: tumour lysis prophylaxis, antimicrobial prophylaxis, management of haemophagocytic lymphohistiocytosis (dexamethasone, etoposide).