Hepatosplenisch T-cel lymfoom

WHO 2022 Hepatosplenic T-cell lymphoma (HSTCL): not confined to the young.

Recent studies have shown that HSTCL is not necessarily a disease of the young; only 49% of patients were younger than 60 years of age in a recent study. Of note, dyspoiesis of bone marrow elements mimicking myelodysplastic syndrome is not uncommon in marrow smears of HSTCL patients, although this does not have any clinical impact. While most cases express TCRγδ (~75%) followed by TCRαβ (~25%), a small subset of cases,

about 5%, are TCR-silent.