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Hepatosplenic T-cell lymphoma
Additional recommended knowledge
SynonymsHepatosplenic γδ T-cell lymphoma[1] ICD-O Code9716/3[1] DefinitionHepatosplenic 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.[1] EpidemiologyThis 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.[2] Clinical FeaturesEtiologyThe 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.[3][4] Clinical PresentationThe typical clinical finding in a patient with hepatosplenic T-cell lymphoma is hepatosplenomegaly.[5] Laboratory FindingsThe constellation of thrombocytopenia, anemia, and leukocytosis is common in patients with hepatosplenic T-cell lymphoma.[2] The discovery of neoplastic cells in peripheral blood is often a finding late in the clinical course.[1] Sites of InvolvementThe spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[2][1] MorphologyThe neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.[2] Spleen and liverThis disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.[1] Bone marrowWhile 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.[1] Peripheral bloodCells of a similar morphology observed in solid organs are observed in peripheral blood.[1] Molecular FindingsImmunophenotypeThe immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.[1][2]
Genetic FindingsClonal 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.[1] Isochromosome 7q has been observed in all cases described so far, sometimes in conjunction with other chromosomal abnormalities such as trisomy 8.[6] References
Categories: Hematology | Types of cancer | Blood disorders |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Hepatosplenic_T-cell_lymphoma". A list of authors is available in Wikipedia. |