Definition
Hepatosplenic T-cell lymphoma (HSTL) is an aggressive subtype of extranodal lymphoma characterized by a clinical presentation with splenomegaly commonly associated with hepatomegaly but without lymphadenopathy and a poor outcome. The neoplasm results from a proliferation of cytotoxic T-cells most often expressing the γδ T-cell receptor (TCR), or less often the αβ TCR. It is usually composed of monomorphic medium-sized lymphoid cells, with marked sinusoidal infiltration of spleen, liver, and bone marrow. It is associated with a recurrent isochromosome 7q cytogenetic abnormality, has a distinct molecular signature, and displays frequent mutations in genes of the JAK-STAT pathway (especially STAT5B) and in the SETD2 gene, encoding a histone methyltransferase.
Up to 20% of HSTL arise in the setting of chronic immune suppression, most commonly long term immunosuppressive therapy for solid organ transplantation or inflammatory bowel...
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References and Further Reading
D’Amore, F., Gaulard, P., Trumper, L., Corradini, P., Kim, W. S., Specht, L., Bjerregaard Pedersen, L., Ladetto, M., & ESMO Guidelines Committee. (2015). Peripheral T-cell lymphomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 26(Suppl 5), v108–v115.
Gaulard, P. Hepatosplenic T-cell lymphoma. In E. S. Jaffe, N. Lee Harris, D. A. Arber, E. Campo, N. L. Harris, & L. Quintanilla-Martinez (Eds.). (2016). Hematopathology (2nd ed., pp. 631–639). Elsevier.
Lemonnier, F., Gaulard, P., & de Leval, L. (2018). New insights in the pathogenesis of T-cell lymphomas. Current Opinion in Oncology, 30(5), 277–284.
McKinney, M., Moffitt, A. B., Gaulard, P., Travert, M., De Leval, L., Nicolae, A., et al. (2017). The genetic basis of hepatosplenic T-cell lymphoma. Cancer Discovery, 7(4), 369–379.
Swerdlow, S., Campo, E., Harris, N. L., Jaffe, E. S., Pileri, S. A., Stein, H., & Thiele, J. (Eds.). (2017). WHO classification of tumours of haematopoietic and lymphoid tissues (IARC WHO classification of Tumours) (Rev. 4th ed.). Lyon: IARC.
Yabe, M., Miranda, R. N., & Medeiros, L. J. (2018). Hepatosplenic T-cell lymphoma: A review of clinicopathologic features, pathogenesis, and prognostic factors. Human Pathology, 74, 5–16.
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Gaulard, P., Lemonnier, F. (2019). Hepatosplenic T-Cell Lymphoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3882-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3882-1
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