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

Kőhalmi, Kinga Viktória; Veszeli, Nóra; Zotter, Zsuzsanna; Benedek, Szabolcs; Imreh, Éva

doi:10.1186/s13023-016-0386-2

Cited by 9 publications

(6 citation statements)

References 22 publications

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“…Attenuated androgens are traditionally used for long‐term prophylaxis of HAE‐1/2 . Androgen derivatives have been demonstrated to be effective in HAE‐1/2, and the oral administration facilitates their use .…”

Section: Therapymentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

467

974

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?

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Section: Therapymentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

467

974

View full text Add to dashboard Cite

show abstract

“…Attenuated androgens are traditionally used for longterm prophylaxis of HAE-1/2 [153][154][155][156][157][158][159][160][161][162]. Androgen derivatives have been demonstrated to be effective in HAE-1/2, and the oral administration facilitates their use [154,156,158].…”

Section: Androgensmentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

World Allergy Organization Journal

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.

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“… 16 , 17 In a recent study, no hematological abnormalities, including erythrocytosis and/or polyglobulia, were detected during long-term danazol treatment. 18 Our observations on patients receiving a very long period of danazol therapy did not revealed hormonal adverse events, as well as hematological or lipid profile abnormalities.…”

Section: Discussionmentioning

confidence: 46%

Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

Akoğlu

Kesim

Yildiz

et al. 2017

An. Bras. Dermatol.

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BackgroundHereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks.ObjectiveWe aimed to investigate the clinical and genetic features of a family with angioedema attacks.MethodsThe medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described.ResultsFive members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke.Study limitationsSmall sample size of participants.ConclusionPatients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

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

Cited by 9 publications

References 22 publications

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update

Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

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