Adult T-cell lymphoma/leukemia - a rare disease in Europe

History and clinical findings: A 45-year-old man was admitted with generali zed itchy papules. He was originally from the Carribean island of Grenada. The disease had started two years before and was diagnosed as lupus erythem atosus, polymorphic light eruption and atopic eczema. Physical examination showed skin-colored papules all over the integument, sebostasis and enlarge d supraclavicular and inguinal lymph nodes. Investigations: Lymphocyte count was elevated with 58% as well as LDH (322 U/I) and gamma GT(133 U/I). In a blood smear characteristic pleomorphic lym phoid cells (flower cells) could be obtained. Histopathologic evaluation de monstrated a subepidermally located infiltrate of pleomorphic lymphocytes w ith epidermal involvement. HTLV-I/II serology (ELISA-screening test) was po sitive. Polymerase chain reaction analysis revealed HTLV-specific sequences . Diagnosis, treatment and course: Diagnosis of adult T-cell lymphoma/leukemi a was obtained. Treatment consisted of interferon alpha (2b) and zidovudine which resulted in a rapid response. However, as a result of an increased l oss of weight (12 kg) this therapy was stopped. Two weeks later generalized lymphadenopathy and disseminated skin lesions were observed. Chemotherapy (CHOP-scheme) was initiated. The patient deceased with signs of an acute le ukemia after a short period. Conclusions: Adult T-cell lymphoma/leukemia is a rare disease in Europe. Ho wever, in patients with skin rashes, and lymphadenopathy, which are origina lly from HTLV-I endemic areas, adult T-cell lymphoma/leukemia should be con sidered.