Hypergammaglobulinemic purpura in cystic fibrosis.

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 Waldenstrom's hypergammaglobulinemic 
purpura. The heterogeneity of this syndrome is discussed. 