Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene

Table 5 Attack frequency in patients with HAE-PLG before and during long-term prophylaxis

Patient No.	Medication	Symptomatic years before LTP	Attacks during symptomatic years before LTP	Years with LTP	Attacks during LTP	Reduction in attack frequency (%)
Progestins*
1	desogestrel	4	75	4	82	0
5	desogestrel	7	432	2	75	0
15	desogestrel	12	135	2	26	0
23	desogestrel	7	83	1	0	100
24	desogestrel	10	210	3	14	77.6
25	desogestrel	6	10	2	0	100
Antifibrinolytics
20	TXA	69	829	4	0	100
26	TXA	7	13	14	0	100
27	TXA	15	17	11	2	81.8
Attenuated androgens**
13	danazol	13	461	8	0	100
20	danazol	58	698	0.3	2	50
27	danazol	15	17	0.3	0	100

C1-INH C1 inhibitor, HAE hereditary angioedema, HAE-PLG HAE with normal C1-INH and the c.988A>G (p.K330E) variant in the PLG gene, LTP long-term prophylaxis, TXA tranexamic acid
*after discontinuing estrogen-containing oral contraceptives
**dose range 100 mg to 200 mg danazol daily

ISSN: 1750-1172