A Pharmacokinetic and Clotting Activity Study of FVIII-PEGLip

Inclusion Criteria: * Male adult patients aged 18 to 60 years; * Severe Haemophilia A (FVIII plasma level \<1IU/dL) with documented history of bleeds (for at least 6 months prior to enrolment); * For patients without inhibitors: inhibitor titre \< 0,6 Bethesda units and no medi-cal history of inhibitors; * For patients with inhibitors: inhibitor titre ≥0,6 Bethesda units or documented medical history of inhibitors titre ≥0,6 Bethesda units; * Adequate hematologic function, defined as platelet count ≥ 100,000/μL and hemoglobin ≥ 8 g/dL (≥ 4.97 mmol/L) at the time of screening; * Adequate hepatic function, defined as total bilirubin ≤ 1.5 × the upper limit of normal (ULN) (excluding Gilbert's syndrome) and aspartate aminotransferase (AST) and alanine aminotransferase (ALT) ≤ 3 ×ULN at the time of screening; no clinical signs or known laboratory/radiographic evidence consistent with cirrho-sis; * Adequate renal function, defined as serum creatinine ≤ 2.5 × ULN and creati-nine clearance by Cockcroft-Gault formula ≥ 30 mL/min; * Patient's written informed consent, confirming his willingness to comply with the requirements of this protocol. Exclusion Criteria: * Low platelet counts (\<100000 / μl); * Congenital or acquired bleeding defects (including acquired hemophilia) other than Hemophilia A; * Abnormal renal function (serum creatinine concentrations greater than 1.3 mg/dL); * Active hepatic disease (persistent aspartate aminotransferase \[AST\] or alanine aminotransferase \[…