Hypergammaglobulinemic purpura in cystic fibrosis

Abstract

Four patients are presented aged 14 to 20 years with cystic fibrosis and recurrent purpura of the legs. They have polyclonal increase of Ig but no intermediate complexes demonstrable by ultracentrifugation. The 4 patients differ from other patients with cystic fibrosis by a rapid deterioration of the clinical condition after the establishment of permanent pulmonary infection, and also by their proneness to Haemophilus influenzae infections. The clinical and laboratory findings are compatible with the diagnosis of Waldenström's hypergammaglobulinemic purpura. The heterogeneity of this syndrome is discussed.