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
Sites of Involvement
The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[5] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001 </ref>
Microscopic Pathology
The neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.
Spleen and liver
This disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.
Bone marrow
While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.
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