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The comments by Marlu et al1 regarding our article on plasminogen activator inhibitor 2 (PAI-2) deficiency in patients with hereditary angioedema with normal C1 inhibitor (HAE-N) is timely and interesting. The very low levels observed in 22 of 23 untreated patients with HAE-N (with or without a factor XII mutation, 4.5 ± 5) were similar to the levels in 11 patients reported in their commentary, but the PAI-2 mean values in normal controls (54 ± 24) and patients with HAE type I (84 ± 52) were over 10 times higher.

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