Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Hepatosplenic T-Cell Lymphoma

Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3882-1



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

  1. 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.CrossRefGoogle Scholar
  2. 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.Google Scholar
  3. 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.PubMedGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.Google Scholar
  6. 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.CrossRefGoogle Scholar

Authors and Affiliations

  1. 1.Département de Pathologie & Inserm U955Hôpital Henri MondorCréteilFrance
  2. 2.Unité Hémopathies Lymphoides & INSERM U955Hôpital Henri MondorCréteilFrance