Hepatosplenic T-cell lymphoma (HSTCL)

2008-07-01   Francesco Cavazzini , Gian Matteo Rigolin , Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Clinics and Pathology

Phenotype stem cell 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.

Bibliography

Pubmed IDLast YearTitleAuthors
89438631996Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin.Cooke CB et al
118079812002Fluorescence 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 et al

Citation

Francesco Cavazzini ; Gian Matteo Rigolin ; Antonio Cuneo

Hepatosplenic T-cell lymphoma (HSTCL)

Atlas Genet Cytogenet Oncol Haematol. 2008-07-01

Online version: http://atlasgeneticsoncology.org/haematological/2099/hepatosplenic-t-cell-lymphoma-(hstcl)

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