The Sézary syndrome with rapid pulmonary dissemination

The authors report the case of a patient with long-standing Sézary syndrome who developed the acute onset of bilateral pulmonary infiltration, severe hypoxemia, and hypotension. Initial diagnostic considerations centered around infection, but an open-lung biopsy revealed "mycosis fungoides" without evidence of an infectious process. The patient showed striking improvement when given vincristine and cyclophosphamide, but ultimately died 3 months later of a nonpulmonary catheter-related infection. This rare clinical association stresses the value of open lung biopsy as a diagnostic measure even in desperately ill individuals.