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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.

Type

Journal article

Journal

Acta Paediatr Scand

Publication Date

07/1978

Volume

67

Pages

443 - 447

Keywords

Adolescent, Adult, Chronic Disease, Cystic Fibrosis, Female, Haemophilus Infections, Haemophilus influenzae, Humans, Leg, Male, Pneumonia, Purpura, Hyperglobulinemic, Sputum