Role of ADAMTS13 deficiency in development of renal microcirculatory tromboses, not associated with thrombotic, thrombocytopenic purpura


N.L. Kozlovskaya, E.Yu. Khafizova, L.A. Bobrova, I.N. Bobkova, A.M. Kuchieva, V.A. Varshavskii, E.S. Stoliarevich, P.V. Avdonin, E.V. Zakharova

Aim. Assessment of activity of ADAMTS13, von Willebrand factor and thrombocytes in patients with thrombophilic states and kidney diseases.
Methods. 20 patients with thrombophilia (genetically based and antiphospholipid syndrome) and kidney diseases (lupus nephritis, chronic glomerulonephritis) with morphological signs of renal thrombotic microngiopathy were included into the study. In all patients serum activity of ADAMTS13 and von Willebrand factor was determined.
Results. Serum activity of ADAMTS13 below the reference (<93%) values (56-92%) was found in 8 (40%) of patients. Serum activity of ADAMTS13 inversly correlated with level of von Willebrand factor (r= -0,3, р<0,05) and directly correlated with thrombocytes blood count (r= 0,57, р<0,01). The most low levels of serum activity of ADAMTS13 were seen in 2 patients with morphological signs of renal acute thrombotic microangiopathy.
Conclusion. Deterioration of ADAMTS13 - von Willebrand factor – thrombocytes cascade can be interpreted as universal mechanism of thombus formation in renal microcirculation.

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