title: The effect of long-term danazol treatment on haematological parameters in hereditary angioedema.
abstract: BACKGROUND
  The 17-alpha-alkylated derivatives of testosterone are often used for the prevention of oedematous episodes in hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE). However, these agents can have many adverse effects, including erythrocytosis and polyglobulia. Our aim was to investigate occurrence of erythrocytosis and polyglobulia after long-term danazol prophylaxis in C1-INH-HAE.
  
  
  METHODS
  During the initial stage of our retrospective study, we explored whether C1-INH-HAE is associated with susceptibility to erythrocytosis and/or polyglobulia. In the second stage, we analyzed the haematological parameters of 39 C1-INH-HAE patients before, as well as after treatment with danazol for 1, 3, or 5 years. In the third stage, we studied the incidence of erythrocytosis and of polyglobulia after dosing with danazol for more than 5 years.
  
  
  RESULTS
  We did not find any significant difference between C1-INH-HAE patients not receiving danazol and healthy controls as regards the occurrence of erythrocytosis or polyglobulia. The haematological parameters did not change after treatment with danazol for 1, 3, or 5 years. Platelet count was an exception-it decreased significantly (p = 0.0115) versus baseline, but within the reference range. Treatment-related polyglobulia did not occur. We observed erythrocytosis in a single female patient after 1-year-and in three female patients after more than 5-year long-treatment with danazol. Erythrocytosis did not require intervention or the discontinuation of danazol therapy.
  
  
  CONCLUSIONS
  We conclude that neither erythrocytosis, nor polyglobulia occurs more often in C1-INH-HAE patients than in healthy individuals; it can be observed only sporadically even after treatment with danazol.
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. kinga.viktoria.kohalmi@gmail.com.
authors.forename: Kinga Viktória
authors.initials: KV
authors.lastname: Kőhalmi
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. veszeli.nora88@gmail.com.
authors.forename: Nóra
authors.initials: N
authors.lastname: Veszeli
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. zotter.zsuzsanna@gmail.com.|Urology Department, Medical Centre, Hungarian Defence Forces, Budapest, Hungary. zotter.zsuzsanna@gmail.com.
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Zotter
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. csuka.dorottya@med.semmelweis-univ.hu.
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. nagysz@kut.sote.hu.
authors.forename: Szabolcs
authors.initials: S
authors.lastname: Benedek
authors.affiliation: Department of Laboratory Medicine, Semmelweis University, Budapest, Hungary. imrehe@kut.sote.hu.
authors.forename: Éva
authors.initials: É
authors.lastname: Imreh
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. vargalilian@gmail.com.
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.affiliation: Hungarian Angioedema Centre, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary. farkas.henriette@med.semmelweis-univ.hu.
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
mesh_terms: D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000235:genetics; D050718:Complement C1 Inhibitor Protein / Q000235:genetics; D003613:Danazol / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D003613:Danazol