PRIMARY CUTANEOUS T-CELL LYMPHOMA - CLINICOPATHOLOGICAL FEATURES AND PROGNOSTIC PARAMETERS OF 35 CASES OTHER THAN MYCOSIS-FUNGOIDES AND CD30-POSITIVE LARGE-CELL LYMPHOMA

Within the group of primary cutaneous T-cell lymphomas (CTCLs), mycosi s fungoides (MF), Sezary's syndrome (SS), and CD30-positive lymphomas have been delineated as clinicopathological entities. Primary CTCLs th at do not belong to one of these entities represent a heterogeneous an d ill-defined group of neoplasms. This paper describes the clinical an d histological features of 35 of such cases. The object of this study was to define prognostic parameters for this group of primary CTCLs. U sing a slightly modified version of the updated Kiel classification, a subdivision was made into CTCL, pleomorphic, small cell type (n=3); p leomorphic, medium-sized cell type (n=6); pleomorphic, large cell type (n=18); and immunoblastic lymphomas (n=8). Altogether, these lymphoma s had a poor prognosis with estimated 2- and 4-year survival rates of 53 and 22 per cent, respectively. Patients with pleomorphic, small and medium-sized cell lymphomas (n=9) proved to have a significantly bett er survival that those with pleomorphic, large cell lymphomas (P=0.032 ) and immunoblastic lymphomas (P=0.008). Primary cutaneous immunoblast ic lymphomas had the worst prognosis with an estimated 2-year survival rate of 14 per cent. Other parameters including age (P=0.345), sex (P =0.662), extent of skin lesions at presentation (P=0.0854), and mode o f initial treatment (P=0.609) had no significant effect on the surviva l time. The results of this study suggest that primary CTCLs other tha n classical MF, SS, and CD30-positive lymphomas have a poor prognosis in most cases, and that the current classification may be a useful mea ns of predicting the clinical behaviour in these lymphomas.