• 1 January 1980
    • journal article
    • research article
    • Vol. 207  (1) , 123-125
Abstract
Finding of an acquired hereditary angioedema[AE]-like syndrome in a patient with myelofibrosis is reported. No previous personal or family history of AE was present. The serum complement pattern showed a marked reduction of C [complement component]1 esterase inhibitor, C1q and C4. All family members had a normal complement profile. Because of frequent attacks of laryngeal AE, prophylactic treatment with danazol was started. A striking clinical response was observed as well as a normalizing effect on the underlying biochemical abnormality.