Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions

Jump to navigation Jump to search
No edit summary
No edit summary
Line 31: Line 31:
|}
|}
==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 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>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>
* 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==
==Gross Pathology==



Revision as of 20:44, 23 February 2016

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.

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]

[3][4] [5]

Gross Pathology

Microscopic Pathology

The characteristic features of neoplastic cells in hepatosplenic T cell lymphoma include:


Microscopic appearance of hepatosplenic T cell lymphoma in different sites
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


Template:WikiDoc Sources