T- and NK-Cell Lymphomas and Systemic Lymphoproliferative Disorders and the Immunodeficiency Setting: 2015 SH/EAHP Workshop Report-Part 4

Dita Gratzinger, Daphne de Jong, Elaine S Jaffe, Amy Chadburn, John K C Chan, John R Goodlad, Jonathan Said, Yasodha Natkunam

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22 Citations (Scopus)


Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review immunodeficiency-related T- and natural killer (NK)-cell lymphoproliferations.

Methods: The Workshop Panel reviewed 88 T- or NK-cell lymphoproliferations and rendered consensus diagnoses.

Results: Hyperplasias of T-cell subsets may be clonal; retained architecture and the clinical setting support a benign diagnosis. Specific associations include hepatosplenic T-cell lymphoma with iatrogenic immunosuppression and breast implants with an indolent variant of anaplastic large cell lymphoma. Epstein-Barr virus (EBV)-positive T-cell lymphomas rarely occur in the acquired immunodeficiency setting. Systemic T- and NK-cell lymphoma of childhood overlaps with chronic active EBV and reversible hemophagocytic lymphohistiocytosis-related T-cell lymphoproliferations.

Conclusions: Immunodeficiencies predispose to T-cell hyperplasias, which must not be overdiagnosed as lymphoma. Many T-cell lymphomas in the immunodeficiency setting are likely coincidental, with specific exceptions. Systemic T- or NK-cell lymphomas are part of a spectrum of EBV+ T or NK lymphoproliferations and can present in the acquired immunodeficiency setting.

Original languageEnglish
Pages (from-to)188-203
Number of pages16
JournalAmerican Journal of Clinical Pathology
Issue number2
Publication statusPublished - 1 Feb 2017


  • Education
  • Epstein-Barr Virus Infections
  • Female
  • Humans
  • Immunologic Deficiency Syndromes
  • Journal Article
  • Lymphoma, Extranodal NK-T-Cell
  • Lymphoma, T-Cell
  • Lymphoproliferative Disorders
  • Male

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