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

Monotonous appearance
Small amount of cytoplasm
Inconspicuous nucleoli

**Microscopic appearance of hepatosplenic T cell lymphoma in different sites**
Sites of involvement	Description
Spleen and liver	Are always involved Distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads
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

↑ ^1.0 ^1.1 Hepatosplenic T cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52ee/. Accessed on February 22, 2016
↑ [1]
↑ 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
↑ [2]
↑ 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

Template:WikiDoc Sources

[cancer.gov-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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