C1 inhibitor (C1-INH) controls protease activity in complement, contact, coagulation and fibrinolytic systems. Its deficiency causes recurrent angioedema of the skin, bowel mucosa and upper airways. Diagnosis of an ongoing attack may be problematic when angioedema affects mucosa which cannot be easily inspected. Since generation of thrombin and activation of fibrinolytic system occur during angioedema in C1-INH deficiency, we used commonly available methods to measure plasma levels of prothrombin fragment F1 + 2 (marker of thrombin generation) and D-Dimer (marker of fibrin degradation). We studied 32 patients (14 men) with C1-INH deficiency. Thirteen patients, 9 with hereditary deficiency (HAE) and 4 with acquired deficiency (AAE), were observed during 24 acute attacks; 22 patients, 19 with HAE and 3 with AAE were observed during remission. Twenty normal subjects (10 men and 10 women; age range 30 to 66 years) served as controls. F1 + 2 was measured by ELISA. D-Dimer was measured by ELISA and a latex method. Results are given as mean ± S.E.M. F1 + 2 was higher in patients in remission than in controls (331 ± 72 pmol/l versus 167 ± 22 pmol/l; p = 0.007). F1 + 2 further increased during angioedema, 1482 ± 222 pmol/l (p = 0.0001). D-Dimer was higher in patients in remission than in controls both with ELISA (1521 ± 544 versus 516 ± 150 ng/ml, p = 0.007) and latex method (544 ± 168 ng/ml versus 111 ± 28 ng/ml, p = 0.006). D-Dimer further increased in patients during angioedema: 2932 ± 1074 ng/ml with ELISA and 800 ± 196 ng/ml with latex method (p = 0.0001 and 0.0001). Plasma levels of F1 + 2 and D-Dimer are significantly increased in patients with C1-INH deficiency. Both parameters further increase during angioedema, but only F1 + 2 levels are clearly demarcated in the two conditions. Therefore, we propose to use plasma levels of F1 + 2 as plasmatic markers to distinguish an ongoing angioedema in patients with C1-INH deficiency.

Plasmatic markers of acute attack in patients with angioedemadue to C1 inhibitor deficiency / A. Zanichelli, M. Cugno, S. Griffini, L. Maggioni, A.G. Bellatorre, L.C. Zingale, M. Cicardi. - In: MOLECULAR IMMUNOLOGY. - ISSN 0161-5890. - 44:16(2007 Sep), pp. 3969-3970. ((Intervento presentato al 11. convegno European meeting on Complement in Human Disease tenutosi a Cardiff nel 2007 [10.1016/j.molimm.2007.06.142].

Plasmatic markers of acute attack in patients with angioedemadue to C1 inhibitor deficiency

M. Cugno;L. Maggioni;M. Cicardi
2007

Abstract

C1 inhibitor (C1-INH) controls protease activity in complement, contact, coagulation and fibrinolytic systems. Its deficiency causes recurrent angioedema of the skin, bowel mucosa and upper airways. Diagnosis of an ongoing attack may be problematic when angioedema affects mucosa which cannot be easily inspected. Since generation of thrombin and activation of fibrinolytic system occur during angioedema in C1-INH deficiency, we used commonly available methods to measure plasma levels of prothrombin fragment F1 + 2 (marker of thrombin generation) and D-Dimer (marker of fibrin degradation). We studied 32 patients (14 men) with C1-INH deficiency. Thirteen patients, 9 with hereditary deficiency (HAE) and 4 with acquired deficiency (AAE), were observed during 24 acute attacks; 22 patients, 19 with HAE and 3 with AAE were observed during remission. Twenty normal subjects (10 men and 10 women; age range 30 to 66 years) served as controls. F1 + 2 was measured by ELISA. D-Dimer was measured by ELISA and a latex method. Results are given as mean ± S.E.M. F1 + 2 was higher in patients in remission than in controls (331 ± 72 pmol/l versus 167 ± 22 pmol/l; p = 0.007). F1 + 2 further increased during angioedema, 1482 ± 222 pmol/l (p = 0.0001). D-Dimer was higher in patients in remission than in controls both with ELISA (1521 ± 544 versus 516 ± 150 ng/ml, p = 0.007) and latex method (544 ± 168 ng/ml versus 111 ± 28 ng/ml, p = 0.006). D-Dimer further increased in patients during angioedema: 2932 ± 1074 ng/ml with ELISA and 800 ± 196 ng/ml with latex method (p = 0.0001 and 0.0001). Plasma levels of F1 + 2 and D-Dimer are significantly increased in patients with C1-INH deficiency. Both parameters further increase during angioedema, but only F1 + 2 levels are clearly demarcated in the two conditions. Therefore, we propose to use plasma levels of F1 + 2 as plasmatic markers to distinguish an ongoing angioedema in patients with C1-INH deficiency.
Settore MED/09 - Medicina Interna
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/63372
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