HEPATOSPLENIC gd T-CELL LYMPHOMA (HTCL) is an uncommon but distinct T-cell lymphoma with a characteristic growth pattern involving the hepatic and splenic red pulp sinusesref1, ref2, ref3, ref4, ref5, ref6, ref7. Most tumors have a gd T-cell phenotype; tumors arising from aß T-cells are clinically and pathologically similarref. Rare cases of HSTCL expressing the aß chain have been reported, but the prognostic relevance is unknownref. The majority of patients succumb to their disease, with an overall median survival of 16 monthsref. The gd phenotype has been rarely identified in primary cutaneous T-cell lymphoma and is a marker of poor prognosis (median survival 15 months vs. 166 months in aß subtypes)ref. Unlike HSTCL, which express TIA1 but are negative for granzyme B and perforin cytotoxic proteins, primary cutaneous gd TCL express all cytotoxic proteins, thus demonstrating an activated T-cell phenotype which may contribute to their aggressive behaviorref. gd T-cells are normally the first line of defense at epidermal and epithelial surfaces and they represent 10-12% of lymphocytes in the spleenref1, ref2
Epidemiology : most cases occur in young adult males (median age, 29-34 yrs)
Aetiology : approximately 20% arise in immunosuppressed, predominantly posttransplant patientsref1, ref2, ref3
Symptoms & signs : B symptoms, splenomegaly (98%), hepatomegaly (80%), no lymphadenopathy, anemia, and severe thrombocytopenia (80-85%).
Laboratory examinations : bone marrow involvement Therapy : most patients are refractory or have brief responses to anthracycline therapy with death occurring < 1 year after diagnosisref1, ref2.
Differential diagnosis :
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