peripheral T-cell lymphoid neoplasm

Introduction

Category includes heterogeneous diseases.

Classification

• working formulation:
  • diffuse small cleaved cell lymphoma
  • large cell immunoblastic lymphoma
  • diffuse mixed small & large cell lymphoma
• Kiel classification:
  • T-zone lymphoma,
  • lymphoepithelioid cell lymphoma

Microscopic pathology

Immunophenotype: T-cell associated antigens variable CD2 +/-, CD3 +/-, CD5 +/- CD7 -/+, CD4 > CD8.

Genetics

TCR usually rearranged, Ig usually germline

Management

• treated with combination chemotherapy

More general terms

• T-cell lymphoid neoplasm (T-cell leukemia, T-cell lymphoma)

More specific terms

• adult T-cell lymphoma/leukemia (ATL/L)
• anaplastic large cell lymphoma (ALCL), CD30+, T-cell type
• angiocentric T-cell lymphoma (lymphomatoid granulomatosis, polymorphic reticulosis, midline malignant reticulocytosis, Stewart's granuloma)
• angioimmunoblastic T-cell lymphoma (AILD)
• cutaneous T-cell lymphoma (CTCL)
• intestinal T-cell lymphoma
• T-cell prolymphocytic leukemia (TPLL)
• unspecified primary cutaneous peripheral T-cell lymphoma