HTLV-1-ASSOCIATED CUTANEOUS DISEASE - A CLINICOPATHOLOGICAL AND MOLECULAR STUDY OF PATIENTS FROM THE UK

The clinicopathological features of eight patients with cutaneous dise ase associated with HTLV-1 infection are reviewed. All were U.K. resid ents of West Indian extraction, and two are currently alive. Disease r emained confined to the skin in two patients. Five patients with a cut aneous prodromal phase developed leukaemia after a median duration of 124 months (3 months-21 years), and in one of these combination chemot herapy produced a sustained clinical remission for 20 months. Two pati ents developed cutaneous disease after remission of their leukaemia. C utaneous lesions were heterogeneous and included localized papules, a generalized papulonodular eruption, diffuse and localized erythematous plaques, pompholyx-like lesions on the palms and soles, and tumours. The histology of the skin lesions was also variable, and consisted of a heavy dermal infiltrate with lymphocytes, histiocytes, plasma cells, eosinophils and cytologically atypical mononuclear cells. Epidermotro pism was present in biopsies from five patients. Tumour cells with lar ge, densely staining, pleomorphic nuclei, arranged in rows between col lagen bundles, were present in the majority of cases. In one patient t he infiltrate also consisted of epithelioid cells and multinucleated g iant cells. Six cases were classified histologically as pleomorphic T- cell lymphoma, and two as cerebriform or mycosis fungoides type. Molec ular studies revealed a clonal T-cell population associated with monoc lonal integration of HTLV- 1 provirus in tissue DNA from six patients. In two patients HTLV- 1 integration was established retrospectively u sing enzymatic in vitro amplification of a specific HTLV-1 pol gene se quence in DNA extracted from paraffin-embedded sections. This study in dicates that the clinical and pathological features of HTLV-1-associat ed cutaneous disease are diverse. Patients may have disease confined t o the skin for prolonged periods, either at presentation or following clinical relapse cutaneous adult T-cell lymphoma. Molecular techniques allow distinction from other types of cutaneous T-cell lymphoma, and provide an opportunity for retrospective studies of archival material.