Yabe et al reported criteria for the diagnosis of hepatosplenic T-cell lymphoma (HSTCL). Large granular T-cell leukemia is an important condition in the differential diagnosis. The authors are from Memorial Sloan Kettering Cancer Center and the University of Texas MD Anderson Cancer Center.
Typical patient: elderly, with indolent course
Findings seen in 100% of patients (absence against the diagnosis):
(1) absence of splenomegaly
(2) monoclonal T-cell receptor gene rearrangement
(3) lymphocytes contain azurophilic granules in cytoplasm
Findings seen in a high percent of patients:
(1) immunophenotype with CD5+, CD8+, CD57+, granzyme B+, TCR-alpha-betta+ (71%)
Findings seen in some cases:
(1) B-symptoms (29%)
(2) peripheral blood lymphocytosis > 5,000 per µL (29%)
(3) immunophenotype: CD3+, CD56+, TIA-1+, TCR-gamma-beta+; CD5-, CD4-, CD8-, granzyme B- (14%)
Findings excluding the diagnosis (0%):
(1) tumor cells expanding the bone marrow sinuses (usually interstitial or intrasinusoidal)
(2) lymphadenopathy
(3) extranodal site of involvement (including liver and spleen)
(4) evidence of infection with EBV, HIV or HTLV-1
(5) isochromosome 7q or trisomy 8
