Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions

Jump to navigation Jump to search
← Older edit
VisualWikitext
No edit summary
Mmir (talk | contribs)
CMEAdmin, CMEUser, Bureaucrats, bureaucrats, nocaptcha, smwadministrator, Administrators, Widget editors
14,850 edits
Mahshid
 
(14 intermediate revisions by 2 users not shown)
Line 4: Line 4:


==Overview==
==Overview==
Hepatosplenic T cell lymphoma arises from 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
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]].
Line 30: Line 29:
|-
|-
|}
|}
==Association==
==Association==
* Hepatosplenic T cell lymphoma is seen more often in 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>
* 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]]
* 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]].
:* Patients with [[inflammatory bowel disease]]
 
==Gross Pathology==
==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==
Line 43: Line 45:
* 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  
Line 77: Line 85:
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]
[[Category:Gastroenterology]]
[[Category:Hepatology]]

Latest revision as of 02:02, 27 November 2017

Hepatosplenic T cell lymphoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Epidemiology and Demographics

Risk Factors

Screening

Differentiating Hepatosplenic T cell lymphoma from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Biopsy

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hepatosplenic T cell lymphoma pathophysiology On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hepatosplenic T cell lymphoma pathophysiology

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hepatosplenic T cell lymphoma pathophysiology

CDC on Hepatosplenic T cell lymphoma pathophysiology

Hepatosplenic T cell lymphoma pathophysiology in the news

Blogs on Hepatosplenic T cell lymphoma pathophysiology

Directions to Hospitals Treating Hepatosplenic T cell lymphoma

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

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


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Hepatosplenic_T_cell_lymphoma_pathophysiology&oldid=1394345"