Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions

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==Overview==
==Overview==
The cell of origin for this disease is an immature cytotoxic [[T-cell]] clonally expressing the γδ [[T-cell receptor]].  
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 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 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
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:* 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 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.<ref>[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8085554&query_hl=10&itool=pubmed_ExternalLink]</ref>
* Hepatosplenic T cell lymphoma is seen more often in
<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>
:* Immunosuppressed solid organ transplant recipients<ref>[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8085554&query_hl=10&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>
:* Patients treated with the immunosuppressants [[azathioprine]], [[infliximab]], and [[adalimumab]]
===Drug Induced===
:* Patients with [[inflammatory bowel disease]]
* Mercaptopurine


==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===
==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
* Small amount of [[cytoplasm]]  
* Small amount of [[cytoplasm]]  
* Inconspicuous [[nucleoli]]
* Inconspicuous [[nucleoli]]
<gallery widths=200px>
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>
</gallery>




{| 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 in different sites
|+ ''' 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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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

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:


Microscopic appearance of hepatosplenic T cell lymphoma
Sites of involvement Description
Spleen and liver
Bone marrow
  • Commonly involved
  • Detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern
  • Immunohistochemistry can aid in the detection
Peripheral blood
  • Cells of a similar morphology observed in solid organs are observed in peripheral blood

References

  1. ↑ 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
  2. ↑ [1]
  3. ↑ 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
  4. ↑ [2]
  5. ↑ 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. ↑ 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


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