Lymphoma & related disorders
WHO classification-T cell

Topic Completed: 1 January 2012

Minor changes: 14 September 2020

Copyright: 2001-2019,, Inc.

PubMed Search: WHO classification of tumors of hematopoietic and lymphoid tissues (T/NK)

Dragos C. Luca, M.D.
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Cite this page: Luca D. WHO classification-T cell. website. Accessed September 24th, 2020.
Principles of Classification
  • Based on all available information (morphology, immunophenotype, genetics, clinical)
  • No one antigenic marker is specific for any neoplasm (except ALK1)
  • Immune profiling less helpful in subclassification of T cell lymphomas then B cell lymphomas
  • Certain antigens commonly associated with specific disease entities but not entirely disease specific
    • CD30: common in anaplastic large cell lymphoma but also classic Hodgkin lymphoma and other B and T cell lymphomas
    • CD56: characteristic for nasal NK/T cell lymphoma, but also other T cell neoplasms and plasma cell disorders
  • Variation of immunophenotype within a given disease (hepatosplenic T cell lymphoma: usually γδ but some are αβ)
  • Recurrent genetic alterations have been identified for many B cell lymphomas but not for most T cell lymphomas
  • No attempt to stratify lymphoid malignancies by grade
  • Recognize the existence of grey zone lymphomas
  • This multiparameter approach has been validated in international studies as highly reproducible
Classification of Tumors of Hematopoietic and Lymphoid Tissues (T/NK)
Precursor T lymphoid neoplasms
  • T lymphoblastic leukemia / lymphoma, 9837/3

Mature T cell and NK cell neoplasms
  • T cell prolymphocytic leukemia, 9834/3
  • T cell large granular lymphocytic leukemia, 9831/3
  • Chronic lymphoproliferative disorder of NK cells, 9831/3
  • Aggressive NK cell leukemia, 9948/3
  • Systemic EBV positive T cell lymphoproliferative disease of childhood, 9724/3
  • Hydroa vacciniforme-like lymphoma, 9725/3
  • Adult T cell leukemia / lymphoma, 9827/3
  • Extranodal NK/T cell lymphoma, nasal type, 9719/3
  • Enteropathy associated T cell lymphoma, 9717/3
  • Hepatosplenic T cell lymphoma, 9716/3
  • Subcutaneous panniculitis-like T cell lymphoma, 9708/3
  • Mycosis fungoides, 9700/3
  • Sézary syndrome, 9701/3
  • Primary cutaneous CD30 positive T cell lymphoproliferative disorders:
    • Lymphomatoid papulosis, 9718/1
    • Primary cutaneous anaplastic large cell lymphoma, 9718/3
  • Primary cutaneous γδ T cell lymphoma, 9726/3
  • Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T cell lymphoma, 9709/3
  • Primary cutaneous CD4 positive small / medium T cell lymphoma, 9709/3
  • Peripheral T cell lymphoma, NOS, 9702/3
  • Angioimmunoblastic T cell lymphoma, 9705/3
  • Anaplastic large cell lymphoma, ALK positive, 9714/3
  • Anaplastic large cell lymphoma, ALK negative, 9702/3

    *Italicized codes and entities are provisional
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