- Email: [email protected]
Homozygous deficiency of protein c with neonatal deep vein thrombosis and new symptoms at young age
Vol. 70, Suppl. 1
ABSTRACTS OF 12TH NATIONAL CONGRESS
C 219 HOMOZYGOUS DEFICIENCY OF PROTEIN C WITH NEONATAL DEEP VEIN THROMBOSIS AND NEW SYMPTOMS AT YOUNG AGE. IOVinO, P.Pola*, V. De Stefano, S. Mastrangelo, M.S. R. Flare*, B. Bizzi, G. Leone. 1st. Semeiotica Medica e ?? Cattedra Metodologia Clinica, Universita Cattolica, Roma. Homozygous deficiency of protein C (PC) is associated with neonatal purpura fulminans. Some homozygotes with recurrent thromboembolism with late onset are described. Here we report the case of a 19 y.o. man with left ilio-femoral and popliteal vein thrombosis. He referred at one month of age right iliofemoral vein thrombosis, angiographically documented, with apparent spontaneous regression. His history was negative until a further episode of right ilio-femoral vein thrombosis occurred at 17 after a trauma. AT III, total and free PS, plasminogen were normal. PC antigen was 16% and activity 12%. Both parents as well as the paternal grand-mother and a paternal aunt had antigen and activity PC levels reduced at same extent (range 49-65%): familial history was free of thrombosis even after risk situations. A probable homozygous PC deficiency was suggested. This case gives further evidence of the clinical heterogeneity of severe PC deficiency. c 220 DECREASE IN INTERLEUKIN 2. gelo, S. Sica, 1st. Semeiotica
PROTEIN C DURING TREATMENT WITH G. Leone, V. De Stefano, S. MastranP. Salutari, E. Ortu, B. Bizzi. Medica, Universita Cattolica, Roma.
Some hemostatic parameters were investigated in 4 patients with acute leukemia in complete remission (2 ALL after autologous transplantation and 2 ANLL, 2 M and 2 F, age 26-56) during treatment with high doses of IL-2 (12 MU/sgm for 5 days). No significat alteration was found in levels of fibrinogen, total and free protein S and clotting activity of factors XII, XI, IX, VIII, VII, x. A significant decrease in AT III activity (nadir 64+27%), protein C activity (nadir 5827%) and plasminogen activity (nadir 81+5%) was found. The reduction was noticed since the 2nd-3rd day of treatment and the nadir value was reached at the 5th-6th day: values comparable to the basal ones were restored after 5-7 days. These data were corrected for the albumin decrease, considered a reliable index of the leakage of the proteins into interstitial fluid; a significant decrease in PC levels was confirmed. The reduction of PC (and AT III) could play a role in the pathogenesis of coronary ischemia reported during IL-2.
s107
ABSTRACTS OF 12TH NATIONAL CONGRESS
C 219 HOMOZYGOUS DEFICIENCY OF PROTEIN C WITH NEONATAL DEEP VEIN THROMBOSIS AND NEW SYMPTOMS AT YOUNG AGE. IOVinO, P.Pola*, V. De Stefano, S. Mastrangelo, M.S. R. Flare*, B. Bizzi, G. Leone. 1st. Semeiotica Medica e ?? Cattedra Metodologia Clinica, Universita Cattolica, Roma. Homozygous deficiency of protein C (PC) is associated with neonatal purpura fulminans. Some homozygotes with recurrent thromboembolism with late onset are described. Here we report the case of a 19 y.o. man with left ilio-femoral and popliteal vein thrombosis. He referred at one month of age right iliofemoral vein thrombosis, angiographically documented, with apparent spontaneous regression. His history was negative until a further episode of right ilio-femoral vein thrombosis occurred at 17 after a trauma. AT III, total and free PS, plasminogen were normal. PC antigen was 16% and activity 12%. Both parents as well as the paternal grand-mother and a paternal aunt had antigen and activity PC levels reduced at same extent (range 49-65%): familial history was free of thrombosis even after risk situations. A probable homozygous PC deficiency was suggested. This case gives further evidence of the clinical heterogeneity of severe PC deficiency. c 220 DECREASE IN INTERLEUKIN 2. gelo, S. Sica, 1st. Semeiotica
PROTEIN C DURING TREATMENT WITH G. Leone, V. De Stefano, S. MastranP. Salutari, E. Ortu, B. Bizzi. Medica, Universita Cattolica, Roma.
Some hemostatic parameters were investigated in 4 patients with acute leukemia in complete remission (2 ALL after autologous transplantation and 2 ANLL, 2 M and 2 F, age 26-56) during treatment with high doses of IL-2 (12 MU/sgm for 5 days). No significat alteration was found in levels of fibrinogen, total and free protein S and clotting activity of factors XII, XI, IX, VIII, VII, x. A significant decrease in AT III activity (nadir 64+27%), protein C activity (nadir 5827%) and plasminogen activity (nadir 81+5%) was found. The reduction was noticed since the 2nd-3rd day of treatment and the nadir value was reached at the 5th-6th day: values comparable to the basal ones were restored after 5-7 days. These data were corrected for the albumin decrease, considered a reliable index of the leakage of the proteins into interstitial fluid; a significant decrease in PC levels was confirmed. The reduction of PC (and AT III) could play a role in the pathogenesis of coronary ischemia reported during IL-2.
s107