Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions
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Image:Hepatosplenic T cell lymphoma peripheral blood smear|Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .<ref name=jmedicalcasereports>Hepatosplenic gamma delta T-cell lymphoma. Journal of medical case reports. http://www.jmedicalcasereports.com/content/7/1/269. Accessed on February 24, 2016 </ref> | Image:Hepatosplenic T cell lymphoma peripheral blood smear|Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .<ref name=jmedicalcasereports>Hepatosplenic gamma delta T-cell lymphoma. Journal of medical case reports. http://www.jmedicalcasereports.com/content/7/1/269. Accessed on February 24, 2016 </ref> | ||
Image:Hepatosplenic T cell lymphoma bone marrow biopsy.jpg|A: Bone marrow biopsy showing cellular marrow with diffuse intrasinusoidal and interstitial lymphoid cell infiltrates (Jenner Giemsa, ×100); B: Arrows indicating intrasinusoidal lymphoid cell infiltrate (Jenner Giemsa, ×400).<ref name=jmedicalcasereports>Hepatosplenic gamma delta T-cell lymphoma. Journal of medical case reports. http://www.jmedicalcasereports.com/content/7/1/269. Accessed on February 24, 2016 </ref> | |||
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Revision as of 15:20, 24 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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Hepatosplenic T cell lymphoma pathophysiology On the Web |
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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. Gamma delta (γδ) T cells constitute 1% to 5% of the circulating lymphocytes in human blood. These cells preferentially home in on some epithelial rich tissues and sinusoidal areas of the splenic red pulp where they represent up to 30% of the whole T-cell population. Hepatosplenic γδ T-cell lymphoma (HSTCL) is a rare aggressive subtype of extra nodal lymphoma.
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.[2]
- Cases of hepatosplenic T-cell lymphoma have been reported in patients treated with the immunosuppressants azathioprine, infliximab, and adalimumab. The majority occurred in patients with inflammatory bowel disease.
Gross Pathology
Microscopic Pathology
The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include:
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Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .[6]
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![A: Bone marrow biopsy showing cellular marrow with diffuse intrasinusoidal and interstitial lymphoid cell infiltrates (Jenner Giemsa, ×100); B: Arrows indicating intrasinusoidal lymphoid cell infiltrate (Jenner Giemsa, ×400).[6]](/images/a/ad/Hepatosplenic_T_cell_lymphoma_bone_marrow_biopsy.jpg)
A: Bone marrow biopsy showing cellular marrow with diffuse intrasinusoidal and interstitial lymphoid cell infiltrates (Jenner Giemsa, ×100); B: Arrows indicating intrasinusoidal lymphoid cell infiltrate (Jenner Giemsa, ×400).[6]
![A: Bone marrow biopsy showing cellular marrow with diffuse intrasinusoidal and interstitial lymphoid cell infiltrates (Jenner Giemsa, ×100); B: Arrows indicating intrasinusoidal lymphoid cell infiltrate (Jenner Giemsa, ×400).[6]](/index.php/File:Hepatosplenic_T_cell_lymphoma_bone_marrow_biopsy.jpg)
| 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
- ↑ 6.0 6.1 Hepatosplenic gamma delta T-cell lymphoma. Journal of medical case reports. http://www.jmedicalcasereports.com/content/7/1/269. Accessed on February 24, 2016