Our results, together with the lack of evidence of chronic inflammation in the C1-INH-HAE patients21, suggest that the high NG counts in these patients may be caused by disturbed adhesion to ECs biasing the ratio between marginated and circulating pools, whereas only a mild activation of circulating NGs occurs as a consequence of more active plasma serine proteases and/or from the elevated soluble E-selectin levels. Here, SELE is linked to hereditary angioedema.