Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
Hepatosplenic T cell lymphoma arises from an immature cytotoxic [[T-cell]] clonally expressing the γδ [[T-cell receptor]]. | |||
==Genetics== | ==Genetics== | ||
* 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> | * 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> | ||
| Line 14: | Line 15: | ||
:* Trisomy 8 | :* Trisomy 8 | ||
===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]]. | ||
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<ref>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</ref><ref>[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=11224598&query_hl=12&itool=pubmed_ExternalLink]</ref> | <ref>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</ref><ref>[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=11224598&query_hl=12&itool=pubmed_ExternalLink]</ref> | ||
<ref>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</ref> | <ref>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</ref> | ||
=== | ==Gross Pathology== | ||
==Microscopic Pathology== | |||
The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include: | The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include: | ||
* Monotonous appearance | * Monotonous appearance | ||
Revision as of 20:40, 23 February 2016
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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
Hepatosplenic T cell lymphoma arises from 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]
Gross Pathology
Microscopic Pathology
The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include:
| Sites of involvement | Description |
|---|---|
| Spleen and liver |
|
| Bone marrow |
|
| 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