Prolonged course of a cutaneous T-cell lymphoma with late systemic involvement

The clinical course of cutaneous T-cell lymphomas is known to be extremely variable. The disease may be present for up to 50 years, although it has a median survival of four to ten years. Clinical manifestations may range from cutaneous involvement alone to widespread systemic involvement. Described here is a patient with an unusually prolonged course of a cutaneous T-cell lymphoma with systemic involvement which reappeared ten and 18 years after the initial presentation. The patient developed Sjögren's syndrome, bone marrow and peripheral nerve involvement late in the course of her disease.