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Editorial
. 2024 Feb 15;143(7):570-571.
doi: 10.1182/blood.2023023292.

Contactless edema via plasmin

Sidney Strickland  1 , Erin H Norris  1
Affiliations
Editorial

Contactless edema via plasmin

Sidney Strickland et al. Blood. .
No abstract available

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Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

Figures

None
The kallikrein-kinin system and HAE. Activation of FXII to FXIIa leads to conversion of plasma prekallikrein (PK) to active plasma kallikrein (PKa). PKa can cleave high- and low-molecular-weight kininogens (K) to release bradykinin. Under normal conditions, C1-INH inhibits FXIIa and prevents propagation of the pathway. Many cases of HAE are caused by insufficient or inactive C1-INH. The article by Dickeson et al shows that a methionine-to-lysine missense mutation in kininogen (K-Lys), identified in a family of patients with HAE, renders it susceptible to cleavage by the fibrinolytic protease plasmin. This mutation thus causes excessive bradykinin formation and can lead to HAE.
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References

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