HEPATOSPLENIC GAMMA-DELTA T-CELL MALIGNANT-LYMPHOMA - REPORT OF THE FIRST CASE IN CHILDHOOD, INCLUDING MOLECULAR MINIMAL RESIDUAL DISEASE FOLLOW-UP

We report the first case of T-cell gamma delta(+) hepatosplenic malign ant lymphoma in childhood. Tumour-specific oligoprobes were developed against the single V1-J1 rearrangement of the delta T-cell receptor (T CR) gene in order to perform minimal residual disease (MRD) studies. M olecular analysis in serial bone marrow samples proved to be of predic tive value concerning the clinical outcome, Clonotypic DNA was not det ected in peripheral blood during the course of the disease until a ref ractory terminal eukaemic phase took place 18 months after the diagnos is. This case demonstrates the usefulness of MRD studies to monitor th e course of disease in at least some subsets of peripheral T-cell lymp homas.