Hepatosplenic T cell lymphoma pathophysiology: Difference between revisions
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{{Hepatosplenic T cell lymphoma}} | {{Hepatosplenic T cell lymphoma}} | ||
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==Overview== | ==Overview== | ||
==Sites of Involvement== | ==Pathophysiology== | ||
===Sites of Involvement=== | |||
The [[spleen]] and [[liver]] are always involved, with [[bone marrow]] involvement frequently present. Nodal involvement is exceedingly rare.[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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> | The [[spleen]] and [[liver]] are always involved, with [[bone marrow]] involvement frequently present. Nodal involvement is exceedingly rare.[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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]]. | The neoplastic cells in this disorder show a monotonous appearance, with a small amount of [[cytoplasm]] and inconspicuous [[nucleoli]]. | ||
===Spleen and liver=== | ====Spleen and liver==== | ||
This disease shows a distinct [[sinusoidal]] pattern of infiltration which spares the splenic [[white pulp]] and hepatic [[portal triads]]. | This disease shows a distinct [[sinusoidal]] pattern of infiltration which spares the splenic [[white pulp]] and hepatic [[portal triads]]. | ||
===Bone marrow=== | ====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]]. | 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=== | ====Peripheral blood==== | ||
Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]]. | Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]]. | ||
==Molecular Findings== | ===Molecular Findings=== | ||
===Immunophenotype=== | ====Immunophenotype==== | ||
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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=== | ===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] | 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> | 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> | ||
Revision as of 16:13, 18 September 2012
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Differentiating Hepatosplenic T cell lymphoma from other Diseases |
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Risk calculators and risk factors for Hepatosplenic T cell lymphoma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]
Overview
Pathophysiology
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.
Molecular Findings
Immunophenotype
The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.
| Status | Antigens |
| Positive | CD3, TCRδ1, TIA-1 |
| Negative | 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.[1]
References