Hepatosplenic T cell lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [4]

Overview

The cell of origin for this disease is an immature cytotoxic T-cell clonally expressing the γδ T-cell receptor.

Genetics

  • Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include:[1]
  • 2-5 copies of i(7)(q10)
  • Isochromosome 7q present
  • Numerical and structural aberrations of the second chromosome 7
  • Rearrangement TRG@, TRB@ genes
  • T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged
  • Trisomy 8

Immunophenotype[1]

The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.

Status Antigens
Positive CD3, TCRδ1, TIA-1, granzyme M, multiple killer immunoglobulin-like receptors (KIR) isoforms
Negative CD4, CD5, CD8, granzyme B, perforin, CD94

Association

Hepatosplenic T cell lymphoma is seen more often in immunosuppressed solid organ transplant recipients, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.[2] [3][4] [5]

Drug Induced

  • Mercaptopurine


Microscopic Pathology

The neoplastic cells in this disorder show a


Microscopic appearance of hepatosplenic T cell lymphoma in different sites
Sites of involvement Description
Spleen and liver
Bone marrow
  • Commonly involved
  • Detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern
  • Immunohistochemistry can aid in the detection
Peripheral blood
  • Cells of a similar morphology observed in solid organs are observed in peripheral blood


References

  1. 1.0 1.1 Hepatosplenic T cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52ee/. Accessed on February 22, 2016
  2. [1]
  3. Ross CW, Schnitzer B, Sheldon S, Braun DK, Hanson CA. "Gamma/delta T-cell posttransplantation lymphoproliferative disorder primarily in the spleen." Am J Clin Pathol. 1994 Sep;102(3):310-5. PMID: 8085554
  4. [2]
  5. Macon WR, Levy NB, Kurtin PJ, Salhany KE, Elkhalifa MY, Casey TT, Craig FE, Vnencak-Jones CL, Gulley ML, Park JP, Cousar JB. "Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas." Am J Surg Pathol. 2001 Mar;25(3):285-96. PMID: 11224598


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