Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions

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Revision as of 13:47, 23 February 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Genetics

Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include:

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

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

Sites of Involvement

The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[3] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001 </ref>

Microscopic Pathology

The neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.

Spleen and liver

This disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.

Bone marrow

While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.

Peripheral blood

Cells of a similar morphology observed in solid organs are observed in peripheral blood.

References

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@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Hepatosplenic T cell lymphoma}}
-{{CMG}}
+{{CMG}}{{AE}} {{AS}}
 ==Overview==
-==Pathophysiology==
+===Genetics===
+* Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include:
+:* 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====
+The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature [[T-cell]].
+{| border="1" cellpadding="5" cellspacing="0"
+|-
+| rowspan="1"| '''Status'''
+| colspan="1" align="center"| '''Antigens'''
+|-
+| rowspan="1"| Positive
+| colspan="1" align="center"| [[CD3]], [[TCRδ1]], [[TIA-1]], [[granzyme M]], multiple killer immunoglobulin-like receptors (KIR) isoforms
+|-
+| rowspan="1"| Negative
+| colspan="1" align="center"| [[CD4]], [[CD5]], [[CD8]], [[granzyme B]], [[perforin]], [[CD94]]
+|-
+|}
 ===Sites of Involvement===
@@ Line 22: / Line 47: @@
 Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]].
-===Molecular Findings===
-====Immunophenotype====
-The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature [[T-cell]].
-{| border="1" cellpadding="5" cellspacing="0"
-|-
-| rowspan="1"| '''Status'''
-| colspan="1" align="center"| '''Antigens'''
-|-
-| rowspan="1"| Positive
-| colspan="1" align="center"| [[CD3]], [[TCRδ1]], [[TIA-1]]
-|-
-| rowspan="1"| Negative
-| colspan="1" align="center"| [[CD4]], [[CD5]], [[CD8]]
-|-
-|}
-===Genetics===
-Clonal rearrangement of the γ gene of the [[T-cell receptor]] is the hallmark of this disease. A few cases have shown rearrangement of the [[T-cell receptor]] β gene. [[Isochromosome]] 7q has been observed in all cases described so far, sometimes in conjunction with other [[chromosomal]] abnormalities such as [[trisomy]] 8.<ref name="alo1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9264394&query_hl=16&itool=pubmed_ExternalLink]
-Alonsozana EL, Stamberg J, Kumar D, Jaffe ES, Medeiros LJ, Frantz C, Schiffer CA, O'Connell BA, Kerman S, Stass SA, Abruzzo LV. "Isochromosome 7q: the primary cytogenetic abnormality in hepatosplenic gammadelta T cell lymphoma." '''Leukemia'''. 1997 Aug;11(8):1367-72. PMID: 9264394</ref>
 ==References==