* 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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Ludovico Coppola, MD; Salvatore Guastafierro, MD; Giovanni Verrazzo, MD; Antonino Coppola, MD; Domenico De Lucia, MD; Angelo Trelli, MD
Accepted for publication February 18, 2002.
From the Department of Gerontology, Geriatric and Metabolic Diseases (Drs L. Coppola, Verrazzo, and A. Coppola), Department of Hematology, Transfusion Medicine, and Transplant Immunology (Drs Guastafierro and Tirelli), and Department of Laboratory Medicine (Dr De Lucia), Second University of Naples, Naples, Italy.
Reprints: Salvatore Guastafierro, MD, Department of Hematology, Second University of Naples, Largo Madonna delle Grazie, 2, 80138 Napoli, Italy (e-mail: email@example.com).
Copyright College of American Pathologists Jul 2002
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