C1 inhibitor infusion modifies platelet activity in hereditary angioedema patients

Archives of Pathology & Laboratory Medicine, Jul 2002 by Coppola, Ludovic, Guastafierro, Salvatore, Verazzo, Giovanni, Coppola, Antonino, Et al

* Context.-Cl inhibitor (Cl-INH) is an alpha^sub 2^-globulin that blocks esterolytic activity of the first component of the classic complement cascade. The alpha-granules of normal human platelets also contain CL-INH, which is expressed on the platelet surface during platelet secretion in healthy patients, but it is clearly reduced in patients with hereditary angioedema (HAE).

Objective.-To evaluate the effects of in vivo C1l-INH concentrate infusion on platelet responsiveness and coagulation system activity in patients with HAE.

Design.-Assessment of the platelet activity and plasma levels of Cl-INH, activated factor XII (XIla), and prothrombin fragment Fl.2 (Fl.2) before and after infusion of 15 U/ kg of Cl-INH concentrate.

Patients.tin 6 patients (4 men and 2 women), HAE was diagnosed according to the accepted clinical and laboratory criteria.

Measurements.-Platelet aggregation (final concentra

tions: adenosine diphosphate, 0.5, 1.25, and 2.5(mu)LM; collagen, 5 (mu)g//mL), CI-INH antigen (radial immunodiffusion), CI-INH activity (chromogenic substrates), and Xlla and Fl.2 (enzyme-linked immunosorbent assay).

Results.-After Cl-INH infusion, we observed a prompt increase of CI-INH level and a slow return toward its plasma preinfusion values within 4 to 7 days, a significant decrease of both adenosine diphosphate- and collagen-induced platelet aggregation versus preinfusion values (maximum after 1-2 days; P

Conclusions.-These data show a role of C1-INH in the control of platelet activity and that its deficiency increases platelet aggregability and plasma levels of Xlla and Fl.2 in patients with HAE.

(Arch Pathol Lab Med. 2002;126:842-845)

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