Yabe et al reported criteria for the diagnosis of hepatosplenic T-cell lymphoma (HSTCL). This is an aggressive form of lymphoma typically seen in adolescents and young adults. The authors are from Memorial Sloan Kettering Cancer Center and the University of Texas MD Anderson Cancer Center.
Sites of involvement: liver, spleen, bone marrow
Findings seen in 100% of patients (absence against the diagnosis):
(1) lymphoma cells expanding the bone marrow sinuses
(2) monoclonal T-cell receptor gene rearrangement
(3) splenomegaly (100%), massive in 76%
Findings seen in a high percent of patients:
(1) B-symptoms (80%)
(3) lymphocytes devoid of azurophilic granules in cytoplasm (93%)
(4) immunophenotype: CD3+, CD56+, TIA-1+, TCR-gamma-beta+; CD5-, CD4-, CD8-, granzyme B- (71%)
Findings seen in some cases:
(1) isochromosome 7q or trisomy 8 (50%)
(2) lymphadenopathy (23%)
Findings seen in a small number of cases:
(1) immunophenotype with CD5+, CD8+, CD57+, granzyme B+, TCR-alpha-betta+ (14%)
(2) peripheral blood lymphocytosis > 5,000 per µL (7%)
Additional factors excluding the diagnosis:
(1) extranodal site of involvement (other than bone marrow, liver and spleen)
(2) infection with EBV, HIV or HTLV-1