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LLs are rare tumours accounting for approximately 2% of all NHLs. More than 85-90% of adult LLs are of T-cell lineage. The leukaemic presentation is most common in children, with predominantly nodal or extranodal infiltration most common in adolescents and young men. Patients commonly present with a rapidly expanding mediastinal tumour. LLs account for less than 5% of adult lymphomas, but over 40% of childhood lymphomas.

T-cell LL tumours comprise lymphoblasts, which are slightly larger than small lymphocytes, with a round nucleus, delicate chromatin, nearly invisible nucleoli and scanty basophilic cytoplasm. The tumour cells have a T-cell phenotype (CD7+, CD3+) and are variably positive for the other T-cell antigens. T-cell receptor gene rearrangement is sometimes observed, but immunoglobulin heavy chain rearrangement is infrequent¹.

References

1. Sweetenham J, & Borowiz M. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004:503-13.

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