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]]. 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. On gross pathology, | 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. Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include isochromosome 7q, TRG@, and T-cell receptor (TCR) Gamma/Delta genes. On gross pathology, diffuse enlargment of [[liver]] and [[spleen]] are characteristic findings of hepatosplenic T cell lymphoma. On microscopic histopathological analysis, monotonous appearance with a small amount of [[cytoplasm]] and inconspicuous [[nucleoli]] are characteristic findings of neoplastic cells of hepatosplenic T cell lymphoma. | ||
==Genetics== | ==Genetics== | ||
* Genes involved in the pathogenesis of hepatosplenic T cell lymphoma | * Genes involved in the pathogenesis of hepatosplenic T cell lymphoma are listed below.<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 | ||
| Line 14: | Line 14: | ||
:* T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged | :* T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged | ||
:* 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>=== | ===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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==Association== | ==Association== | ||
* Hepatosplenic T cell lymphoma is seen more often in | * Hepatosplenic T cell lymphoma is seen more often in | ||
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==Gross Pathology== | ==Gross Pathology== | ||
* Spleen is enlarged with diffuse involvement of the red pulp | * Spleen is enlarged with diffuse involvement of the red pulp and absence of any gross lesions | ||
* Diffuse hepatic enlargement is present | * Diffuse hepatic enlargement is present | ||
==Microscopic 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 | ||
* Small amount of [[cytoplasm]] | * Small amount of [[cytoplasm]] | ||
* Inconspicuous [[nucleoli]] | * Inconspicuous [[nucleoli]] | ||
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{| style="border: 0px; font-size: 90%; margin: 3px;" align=center | {| style="border: 0px; font-size: 90%; margin: 3px;" align=center | ||
|+ ''' Microscopic appearance of hepatosplenic T cell lymphoma | |+ ''' Microscopic appearance of hepatosplenic T cell lymphoma | ||
! style="background: #4479BA; color:#FFF;" | Sites of involvement | ! style="background: #4479BA; color:#FFF;" | Sites of involvement | ||
! style="background: #4479BA; color:#FFF;" | Description | ! style="background: #4479BA; color:#FFF;" | Description | ||
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Latest revision as of 02:02, 27 November 2017
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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. Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include isochromosome 7q, TRG@, and T-cell receptor (TCR) Gamma/Delta genes. On gross pathology, diffuse enlargment of liver and spleen are characteristic findings of hepatosplenic T cell lymphoma. On microscopic histopathological analysis, monotonous appearance with a small amount of cytoplasm and inconspicuous nucleoli are characteristic findings of neoplastic cells of hepatosplenic T cell lymphoma.
Genetics
- Genes involved in the pathogenesis of hepatosplenic T cell lymphoma are listed below.[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][3][4][5]
- Patients treated with the immunosuppressants azathioprine, infliximab, and adalimumab
- Patients with inflammatory bowel disease
Gross Pathology
- Spleen is enlarged with diffuse involvement of the red pulp and absence of any gross lesions
- Diffuse hepatic enlargement is present
Microscopic Pathology
The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include:
-
Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .[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]](/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 |
|
| 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
- ↑ 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