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. 1985 Aug;76(2):413–419. doi: 10.1172/JCI111987

Activation of factor XII-dependent pathways in human plasma by hematin and protoporphyrin.

C G Becker, M Wagner, A P Kaplan, M Silverberg, R W Grady, H Liem, U Muller-Eberhard
PMCID: PMC423828  PMID: 4031058

Abstract

Intravenous administration of hematin is effective in the treatment of acute exacerbations of the inducible porphyrias. In the course of such treatment, coagulopathies have occurred that are characterized by prolongation of prothrombin time, partial thromboplastin time, and formation of fibrin split products. In experiments in vitro with normal human plasma, we observed that hematin and protoporphyrin activated Factor XII-dependent pathways of coagulation and fibrinolysis, and that they generated kallikrein activity. Incubation of protoporphyrin with purified Factor XII resulted in activation as measured by amidolysis of a chromogenic substrate. Neither coproporphyrin, uroporphyrin, delta-aminolevulinic acid, porphobilinogen, or bilirubin activated Factor XII-dependent pathways. Exposure of serum containing added uroporphyrin, coproporphyrin, and protoporphyrin, but not hematin, to ultraviolet light (405 nm) resulted in activation of the classical pathway of the complement system. On the other hand, exposure of plasma containing uroporphyrin or coproporphyrin to ultraviolet light did not result in activation of Factor XII-dependent pathways.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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