| Phenotype / cell stem origin |
This lymphoma entity originates from T-lymphocytes expressing the Gamma Delta subunits of the TCR. Rarely, cases expressing the Alpha Beta subunits were reported. |
| Epidemiology | Young males are affected predominantly. |
| Clinics | The disease presents with hepatosplenomegaly, in the absence of lymphadenopathy. Bone marrow involvement and cytopenias are frequently encountered (Cooke et al., 1996). |
| Pathology | The proliferation consists of medium-sized lymphocytes with a rim of pale cytoplasm. The nuclei show condensed chromatin with inconspicuous nucleoli (Feldman et al., 2006). Typically these cells show an intrasinusoidal pattern of growth sparing the portal triads and the white pulp. Intrasinusoidal bone marrow involvement may occur. The neoplastic lymphocytes are CD3+, CD4- and may express CD8 and CD56. These features, along with negativity for granzyme B and for perforin, indicate a proliferation of Gamma delta resting T lymphocyte. |
| Treatment | Multiagent chemotherapy, including anthracyclines is the treatment of choice. Autologous or allogeneic transplantation may have a role in selected patients (Cooke et al., 1996). |
| Prognosis | The patients usually respond to chemotherapy, but relapses occur frequently and median survival is around three years. Allogeneic BMT may cure some patients. |
| Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. |
| Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES. |
| Blood 1996; 88(11): 4265-4274. |
| PMID 8943863 |
| |
| Classification and histopathology of the lymphomas. |
| Feldman A, Pittaluga S, Jaffe ES. |
| In: Canellos GP, Lister TA, Young BD: The Lymphomas 2nd edition. Saunders Elsevier, Philadelphia, 2006, pp 2-38 |
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| Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression. |
| Wlodarska I, Martin-Garcia N, Achten R, De Wolf-Peeters C, Pauwels P, Tulliez M, de Mascarel A, Briere J, Patey M, Hagemeijer A, Gaulard P. |
| Genes Chromosomes Cancer. 2002; 33: 243-251. |
| PMID 11807981 |
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