Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions

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Revision as of 14:49, 26 February 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [3]

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]

^[3]

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 without any gross lesions
Diffuse hepatic enlargement is present as well

Microscopic Pathology

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

Monotonous appearance
Small amount of cytoplasm
Inconspicuous nucleoli

Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .[4]

Peripheral blood smear showing large atypical lymphoid cells with indented nuclei .^[4]
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).^[4]

**Microscopic appearance of hepatosplenic T cell lymphoma in different sites**
Sites of involvement	Description
Spleen and liver	Are always involved Distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads
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

↑ ^1.0 ^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]
↑ 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
↑ ^4.0 ^4.1 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

[cancer.gov-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] 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

[jmedicalcasereports-4] 4.0 ^4.1 Hepatosplenic gamma delta T-cell lymphoma. Journal of medical case reports. http://www.jmedicalcasereports.com/content/7/1/269. Accessed on February 24, 2016

[1]

[2]

[3]

[4]

@@ Line 32: / Line 32: @@
 ==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>
+:* 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>
 :* Patients treated with the immunosuppressants [[azathioprine]], [[infliximab]], and [[adalimumab]]