Abstract
This rare disorder was initially listed in the REAL classification as a provisional malignancy, because very few reports had been published at that time, but there is certainly now convincing evidence that it is a distinctive sub-type of lymphoma. It was initially described by Farcet and colleagues in 1990 as a “hepatosplenic T-cell lymphoma”.1 They identified several patients presenting with striking hepatosplenomegaly and a unique type of T cell lymphoma.
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References
Farcet JP, Gaulard P, Marolleau J et al. Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor γδ. Blood 1990; 75: 2213–9.
Cooke CB, Krenacs L, Stetler-Stevenson M et al. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic γδ T-cell origin. Blood 1996; 88:4265–74.
Wang CC, Tien HF, Lin MT et al. Consistent presence of isochromosome 7q in hepatosplenic T gamma/delta lymphoma: a new cytogenetic-clinicopathologic entity. Genes Chromosom Cancer 1995; 12: 161–4.
Alonsozana EL, Stamberg J, Kumar D et al. Isochromosome 7q: the primary cytogenetic abnormality in hepatosplenic γδ T-cell lymphoma. Leukemia 1997; 11:1367–72.
Further Reading
Burg G, Dummer R, Wilhelm M et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med 1991; 325: 1078–81.
Ross CW, Schnitzer B, Sheldon S, Braun DK, Hanson CA. Gamma/delta T-cell post transplantation lymphoproliferative disorder primarily in the spleen. Am J Clin Pathol 1994; 102:310–5.
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© 1999 Springer-Verlag London
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Jaffe, E.S. (1999). Hepatosplenic gamma delta T cell lymphoma. In: Mason, D.Y., Harris, N.L. (eds) Human Lymphoma: Clinical Implications of the REAL Classification. Springer, London. https://doi.org/10.1007/978-1-4471-0857-3_35
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DOI: https://doi.org/10.1007/978-1-4471-0857-3_35
Publisher Name: Springer, London
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