Acquired angioedema and hypocompleentemia in a patient with myelofibrosis. Effect of danazol treatment.
This paper reports the findings of an "acquired" hereditary angioedema-like syndrome in a patient with myelofibrosis. No previous personal or family history of angioedema was present. The serum complement pattern showed a marked reduction of Cl esterase inhibitor, Clq and C4. All family members had a normal complement profile. Because of frequent attacks of laryngeal angioedema, prophylactic treatment with danazol was started. A striking clinical response was observed as well as a normalizing effect on the underlying biochemical abnormality.
D000368:Aged; D000799:Angioedema / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D003172:Complement C1 / Q000032:analysis; D003174:Complement C1 Inactivator Proteins / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D003613:Danazol / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D011245:Pregnadienes / Q000627:therapeutic use*; D055728:Primary Myelofibrosis / Q000150:complications*
D003172:Complement C1; D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4; D011245:Pregnadienes; D011415:Complement Factor B; D003613:Danazol