| Abstract: | Within the group of primary cutaneous T-cell lymphomas (CTCLs), mycosis fungoides (MF), Sézary's syndrome (SS), and CD30-positive lymphomas have been delineated as clinicopathological entities. Primary CTCLs that do not belong to one of these entities represent a heterogeneous and ill-defined group of neoplasms. This paper describes the clinical and histological features of 35 of such cases. The object of t his study was to define prognostic parameters for this group of primary CTCLs. Using a slightly modified version of the updated Kiel classification, a subdivision was made into CTCL, pleomorphic, small cell type (n = 3); plemorphic, medium-sized cell type (n = 6); pleomorphic, large cell type (n = 18); and immunoblastic lymphomas (n = 8). Altogether, these lymphomas 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 better survival that those with pleomkorphic, large cell lymphomas (P = 0·032) and immunoblastic lymphomas (P = 0·008). Primary cutaneous immunoblastic 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 = 0·345), sex (P = 0·662), extent of skin lesions at presentation (P = 0·0854), and mode of initial treatment (P = 0·609) had no significant effect on the survival time. The results of this study suggest that primary CTCLs other than classical MF, SS, and CD30-positive lymphomas have a poor prognosis in most cases, and that the current classification may be a useful means of predicting the clinical behaviour in these lymphomas. |
