|
|
| Line 1: |
Line 1: |
| | '''For patient information, click [[Hepatosplenic T cell lymphoma (patient information)|here]]''' |
| | |
| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} | | | Name = {{PAGENAME}} | |
| Line 13: |
Line 15: |
| MeshID = | | | MeshID = | |
| }} | | }} |
| {{SI}} | | {{Hepatosplenic T cell lymphoma}} |
| {{EH}} | | {{CMG}} |
|
| |
|
| ===Synonyms===
| | '''''Synonyms and Keywords:''''' Hepatosplenic γδ T-cell lymphoma |
| Hepatosplenic γδ T-cell lymphoma<ref name="who1"/> | |
|
| |
|
| ===ICD-O Code=== | | ==[[Hepatosplenic T cell lymphoma overview|Overview]]== |
| 9716/3<ref name="who1"/>
| |
|
| |
|
| ===Definition=== | | ==[[Hepatosplenic T cell lymphoma classification|Classification]]== |
| '''Hepatosplenic T-cell lymphoma''' is a systemic [[neoplasm]] comprised of medium-sized cytotoxic [[T-cells]] that show a significant sinusoidal infiltration in the [[liver]], [[spleen]], and [[bone marrow]].<ref name="who1"/>
| |
|
| |
|
| ==Epidemiology== | | ==[[Hepatosplenic T cell lymphoma historical perspective|Historical Perspective]]== |
| This [[lymphoma]] is rare, comprising less than 5% of all cases, and is most common in young adults and adolescents. A distinct male gender preference has been described.<ref name="coo1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8943863&query_hl=7&itool=pubmed_ExternalLink]
| |
| Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES. "Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin." '''Blood'''. 1996 Dec 1;88(11):4265-74. PMID: 8943863</ref>
| |
|
| |
|
| ==Clinical Features== | | ==[[Hepatosplenic T cell lymphoma pathophysiology|Pathophysiology]]== |
|
| |
|
| ===Etiology=== | | ==[[Hepatosplenic T cell lymphoma epidemiology and demographics|Epidemiology & Demographics]]== |
| The cell of origin for this disease is an immature cytotoxic [[T-cell]] clonally expressing the γδ [[T-cell receptor]]. This disease 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 name="ros1"> [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8085554&query_hl=10&itool=pubmed_ExternalLink]
| |
| 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 name="mac1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=11224598&query_hl=12&itool=pubmed_ExternalLink]
| |
| 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>
| |
|
| |
|
| ===Clinical Presentation=== | | ==[[Hepatosplenic T cell lymphoma epidemiology and demographics|Risk Factors]]== |
| The typical clinical finding in a patient with hepatosplenic T-cell lymphoma is [[hepatosplenomegaly]].<ref name="far1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2140703&query_hl=14&itool=pubmed_ExternalLink] Farcet JP, Gaulard P, Marolleau JP, Le Couedic JP, Henni T, Gourdin MF, Divine M, Haioun C, Zafrani S, Goossens M, ''et al''. "Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta." '''Blood'''. 1990 Jun 1;75(11):2213-9. PMID: 2140703</ref>
| |
|
| |
|
| ===Laboratory Findings=== | | ==[[Hepatosplenic T cell lymphoma screening|Screening]]== |
| The constellation of [[thrombocytopenia]], [[anemia]], and [[leukocytosis]] is common in patients with hepatosplenic T-cell lymphoma.<ref name="coo1"/> The discovery of neoplastic cells in peripheral blood is often a finding late in the clinical course.<ref name="who1"/>
| |
|
| |
|
| ===Sites of Involvement=== | | ==[[Hepatosplenic T cell lymphoma causes|Causes]]== |
| The [[spleen]] and [[liver]] are always involved, with [[bone marrow]] involvement frequently present. Nodal involvement is exceedingly rare.<ref name="coo1"/><ref name="who1">[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>
| |
|
| |
|
| ==Morphology== | | ==[[Hepatosplenic T cell lymphoma differential diagnosis|Differentiating Hepatosplenic T cell lymphoma from other Diseases]]== |
| The neoplastic cells in this disorder show a monotonous appearance, with a small amount of [[cytoplasm]] and inconspicuous [[nucleoli]].<ref name="coo1"/>
| |
|
| |
|
| ===Spleen and liver=== | | ==[[Hepatosplenic T cell lymphoma natural history|Natural History, Complications & Prognosis]]== |
| This disease shows a distinct [[sinusoidal]] pattern of infiltration which spares the splenic [[white pulp]] and hepatic [[portal triads]].<ref name="who1"/>
| |
|
| |
|
| ===Bone marrow=== | | ==Diagnosis== |
| 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]].<ref name="who1"/>
| | [[Hepatosplenic T cell lymphoma history and symptoms|History & Symptoms]] | [[Hepatosplenic T cell lymphoma physical examination|Physical Examination]] | [[Hepatosplenic T cell lymphoma staging|Staging]] | [[Hepatosplenic T cell lymphoma laboratory tests|Lab Tests]] | [[Hepatosplenic T cell lymphoma electrocardiogram|Electrocardiogram]] | [[Hepatosplenic T cell lymphoma chest x ray|Chest X Ray]] | [[Hepatosplenic T cell lymphoma CT|CT]] | [[Hepatosplenic T cell lymphoma MRI|MRI]] | [[Hepatosplenic T cell lymphoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Hepatosplenic T cell lymphoma other imaging findings|Other Imaging Findings]] | [[Hepatosplenic T cell lymphoma other diagnostic studies|Other Diagnostic Studies]] |
|
| |
|
| ===Peripheral blood=== | | ==Treatment== |
| Cells of a similar morphology observed in solid organs are observed in peripheral [[blood]].<ref name="who1"/>
| | [[Hepatosplenic T cell lymphoma medical therapy|Medical Therapy]] | [[Hepatosplenic T cell lymphoma surgery|Surgery]] | [[Hepatosplenic T cell lymphoma primary prevention|Primary Prevention]] | [[Hepatosplenic T cell lymphoma secondary prevention|Secondary Prevention]] |
| | {{Hematological malignancy histology}} |
|
| |
|
| ==Molecular Findings==
| |
|
| |
| ===Immunophenotype===
| |
| The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature [[T-cell]].<ref name="who1"/><ref name="coo1"/>
| |
|
| |
| {| 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]]
| |
| |-
| |
| |}
| |
|
| |
| ===Genetic Findings===
| |
| 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.<ref name="who1"/> [[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==
| |
| <div class="references-small"><references/></div>
| |
|
| |
| {{Hematological malignancy histology}}
| |
| {{SIB}}
| |
| [[Category:Hematology|Hepatosplenic T-cell lymphoma]] | | [[Category:Hematology|Hepatosplenic T-cell lymphoma]] |
| [[Category:Types of cancer|Hepatosplenic T-cell lymphoma]] | | [[Category:Types of cancer|Hepatosplenic T-cell lymphoma]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| | [[Category:Disease]] |
| | [[Category:Gastroenterology]] |
| | [[Category:Hepatology]] |
| | |
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |