Fatal disseminated aspergillosis in a previously well young adult with cystic fibrosis.

Patients with cystic fibrosis (CF) are often colonized by Aspergillus species in their respiratory tract but rarely develop infection with this fungus. A young woman with CF who had mild pulmonary involvement and who was in good health developed a fulminant illness presenting as brainstem infarction and simulating thrombotic thrombocytopenic purpura. At necropsy she was found to have disseminated aspergillosis and a depletion of lymphocytes in the thymus-independent zone of splenic follicles. This unique case may be an unusual expression of abnormal T-cell function and the paradoxical histopathologic findings may relate to the disturbance in humoral immunity in children with CF recently described by Matthews et al.