Rare process with inherited abnormal fibrin molecular resulting in a defective fibrin clot formation.
Patients may be asymptomatic or experience life threatening disease.
Of patients with congenital disease: 40% of patients are asymptomatic. 50% have a bleeding process and 10% have a thrombotic or combined thrombotic and bleeding disorder.
Acquired disease usually related to liver disease with up to 50% of patients with severe cirrhosis, hepatoma or hepatitis having bleeding complications.
Acquired disease occurs most often in patients with severe liver disease.
With liver disease a structural defect manifested by increased carbohydrate content with impaired polymerization of fibrin.
Rarely associated with malignancy with primary or metastatic liver tumors.
Acquired disease may be associated with renal cell carcinoma.