Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions
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===Microscopic Pathology=== | ===Microscopic Pathology=== | ||
The neoplastic cells in | The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include: | ||
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* Small amount of [[cytoplasm]] | * Small amount of [[cytoplasm]] | ||
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* Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]] | * Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]] | ||
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==References== | ==References== | ||
Revision as of 20:38, 23 February 2016
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Hepatosplenic T cell lymphoma Microchapters |
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Differentiating Hepatosplenic T cell lymphoma from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Hepatosplenic T cell lymphoma pathophysiology On the Web |
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Risk calculators and risk factors for Hepatosplenic T cell lymphoma pathophysiology |
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 characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include:
| Sites of involvement | Description |
|---|---|
| Spleen and liver |
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| Bone marrow |
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| Peripheral blood |
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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