Acquired Factor X Deficiency in a Patient with Amyloidosis

Abstract

SummaryA patient with extensive amyloidosis and a selective factor X deficiency is described. The following observations indicate that the factor X deficiency in this case is not inherited.1. The first symptoms of a bleeding tendency appeared at an age of 50 years.2. The patient’s four children had no clotting defect.3. After infusion of 1 liter of fresh plasma no increased factor X activity was observed. No anticoagulants could be demonstrated in vitro.

Coagulopathy in a patient with nephrotic syndrome

“…Alternatively, amyloid fibrils selectively adsorb fibrinolysis inhibitory factor(s), thus increases fibrinolysis [10]. Vascular infiltration by amyloid fibrils also prolong bleeding time and increase capillary fragility [11].…”

“…The treatment of patients with amyloidosis and coagulopathy involves supportive measures, but the coagulopathy generally does not respond well to vitamin K or plasma infusion [11,18]. Level of factor X may improve spontaneously [24], or increase after treatments such as plasma exchange [25], melphalan and prednisone [26], splenectomy [19,20], or autologous stem cell transplantation [5].…”

Hemorrhage Because of Amyloid-related Factor X Deficiency after Insertion of Tenckhoff Catheter

“…Elevated thromboplastin time and prothrombin time are the coagulation abnormalities most commonly seen. A few patients also have factor X deficiency, thought to occur through selective binding of factor X to amyloid fibrils in the liver and spleen (5,6).…”

“…Diagnosis can be challenging in patients on PD in view of the broad differential diagnosis for a presentation of abdominal pain and the difficulties of biochemical testing in uremic patients secondary to impaired pancreatic enzyme clearance (3). Furthermore, use of the glucose polymer icodextrin interferes with the serum amylase assay, resulting in falsely low amylase levels (4,5). Here, we present a case of recurrent pancreatitis in a patient on PD using icodextrin.…”

Cardiovascular Complications in Patients with AL Amyloidosis

“…Other coagulation abnormalities such as factor X deficiency, enhanced fibrinolysis, and amyloid angiopathy seem to correlate better with clinical symptoms (15). Deficiencies in specific coagulation factors in have long been recognized and along with factor X, acquired deficiencies in factor IX, factor II and factor VII have also been described (16). Hypofibrinogenemia has also been observed in systemic AL amyloidosis in association with disseminated intravascular coagulation and increased fibrinolysis (17).…”