LYMPHOMATOID PAPULOSIS IN ASSOCIATION WITH MYCOSIS-FUNGOIDES - A STUDY OF 15 CASES

We report clinical findings in 15 patients with lymphomatoid papulosis (LyP) associated with mycosis fungoides (MF). LyP either preceded (n = 4), followed (n = 5) or occurred concurrently with the MF lesions (n = 6). Twenty-eight LyP lesions were classified histologically and ana lysed further with immunostaining for CD3 and CD30. Five biopsies cont ained a predominance of type A cells, six biopsies contained a predomi nance of type B cells, and six were mixed (A + B). However, II biopsie s contained a population of atypical mononuclear cells with large hype rchromatic nuclei that we have termed indeterminate cells. These cells contained a thin rim of eosinophilic cytoplasm and showed strong CD30 but absent, faint or normal CD3 staining. In seven biopsies from five separate patients these cells represented the predominant cell type a nd we have termed this the pleomorphic variant of LyP. Analysis of T-c ell receptor genes using Southern blot analysis and polymerase chain r eaction/single strand conformational polymorphism analysis identified a T-cell clone in six of 16 LyP lesions and nine of 16 MF lesions. In the three patients who had clones in both types of skin lesions, the c lones were identical. Only two of 10 blood samples, both of which were from the same patient, had a T-cell clone and none of two lymph nodes showed evidence of a clonal population. To date all patients are aliv e with a median follow-up of 15 years from the onset of the first lesi on. One patient has developed an anaplastic large cell lymphoma of the nasopharynx. These data augment the current literature on the associa tion of LyP and MF and suggest that the association usually carries a favourable prognosis.