FANCONIS ANEMIA ASSOCIATED WITH MULTICENTRIC BOWENS-DISEASE AND DECREASED NK CYTOTOXICITY

We report a patient with Fanconi's anaemia and multiple lesions of vul vo-anal Bowen's disease. She was thrombocytopenic and lymphopenic, and NK-mediated cytotoxicity was undetectable. The vulvar lesions did not contain papillomavirus DNA. In vitro studies showed a possible benefi t from acitretin treatment on bone marrow stem cells. However, low-dos e acitretin given for 14 months did not prevent the development of an anal squamous carcinoma. Acitretin therapy was, however, associated wi th a sustained rise in the platelet count.