Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions
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==Genetics== | ==Genetics== | ||
* Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include: | * Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include:<ref name=cancer.gov> Hepatosplenic T cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52ee/. Accessed on February 22, 2016</ref> | ||
:* 2-5 copies of i(7)(q10) | :* 2-5 copies of i(7)(q10) | ||
:* Isochromosome 7q present | :* Isochromosome 7q present | ||
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:* Trisomy 8 | :* Trisomy 8 | ||
====Immunophenotype==== | ====Immunophenotype<ref name=cancer.gov> Hepatosplenic T cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52ee/. Accessed on February 22, 2016</ref>==== | ||
The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature [[T-cell]]. | The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature [[T-cell]]. | ||
Revision as of 13:50, 23 February 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
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 |
Sites of Involvement
The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[3] 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