The effect of long-term danazol treatment on haematological parameters in hereditary angioedema Rare immune deficiencies

Kinga Viktória Kohalmi, Nóra Veszeli, Zsuzsanna Zotter, Dorottya Csuka, Szabolcs Benedek, Éva Imreh, L. Varga, H. Farkas

Research output: Contribution to journalArticle

6 Citations (Scopus)

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.

Original languageEnglish
Article number18
JournalOrphanet Journal of Rare Diseases
Volume11
Issue number1
DOIs
Publication statusPublished - Feb 25 2016

Fingerprint

Hereditary Angioedemas
Danazol
Polycythemia
Therapeutics
Platelet Count
Testosterone
Reference Values
Retrospective Studies
Incidence

Keywords

  • C1-inhibitor deficiency
  • danazol
  • Erythrocytosis
  • Haematology
  • Hereditary angioedema
  • Polyglobulia
  • Prophylaxis

ASJC Scopus subject areas

  • Medicine(all)
  • Genetics(clinical)
  • Pharmacology (medical)

Cite this

The effect of long-term danazol treatment on haematological parameters in hereditary angioedema Rare immune deficiencies. / Kohalmi, Kinga Viktória; Veszeli, Nóra; Zotter, Zsuzsanna; Csuka, Dorottya; Benedek, Szabolcs; Imreh, Éva; Varga, L.; Farkas, H.

In: Orphanet Journal of Rare Diseases, Vol. 11, No. 1, 18, 25.02.2016.

Research output: Contribution to journalArticle

Kohalmi, Kinga Viktória ; Veszeli, Nóra ; Zotter, Zsuzsanna ; Csuka, Dorottya ; Benedek, Szabolcs ; Imreh, Éva ; Varga, L. ; Farkas, H. / The effect of long-term danazol treatment on haematological parameters in hereditary angioedema Rare immune deficiencies. In: Orphanet Journal of Rare Diseases. 2016 ; Vol. 11, No. 1.
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AU - Zotter, Zsuzsanna

AU - Csuka, Dorottya

AU - Benedek, Szabolcs

AU - Imreh, Éva

AU - Varga, L.

AU - Farkas, H.

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