Successful management of bleeding in a patient with factor V inhibitor by platelet transfusions

Abstract

A 71-yr-old male was seen because of the development of severe bleeding diathesis secondary to an acquired factor V inhibitor. The lack of clinical and laboratory response to fresh frozen plasma prompted us to treat him with platelet transfusions during 4 separate bleeding episodes; on each occasion he received 1 0-1 5 U of homologous platelet concentrates. There was a remarkable clinical response associated with a marked correction of the prolonged prothrombin time. prolonged partial thromboplastin time. and prolonged Russell viper venom time; this correlated with higher factor V levels and complete neutralization of the inhibitor. The beneficial effect of platelet transfusion lasted 5-6 days. The inhibitor was of low titer. and it was precipitated by staphylococcal protein A. The in vivo response to platelet transfusion correlated with subsequently performed in vitro experiments. Nonwashed control platelet pellets mixed with inhibitor plasma partially corrected the prolonged PTT. whereas washed platelets or albumin density gradient separated

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

“…It has been shown that in acquired FV deficiency due to the development of FV autoantibodies, platelet FV is relatively protected from antibody neutralization [65]. Platelet infusions have been reported to be effective in acquired FV deficiency [66] and may therefore also be an effective treatment alternative in patients with hereditary deficiency complicated by inhibitors. Prophylactic use of weekly platelet infusions was effective in one inhibitor patient in stabilizing a subdural haematoma [57].…”

Guideline for the diagnosis and management of the rare coagulation disorders

“…Symptomatic FV inhibitors in F5D have been neutralized using large volumes of FFP (Di Paola et al, 2001) or intravenous immunoglobulin (Nesheim et al, 1986). Platelet transfusion was effective in acquired FV deficiency not related to F5D (Chediak et al, 1980). A specific FV concentrate (Kedrion Bipharma, Barga, Italy) is currently in clinical development.…”

Response of factor V inhibitor to rituximab in a patient who received liver transplantation for primary biliary cirrhosis

“…The patient reported here presented with mild bleeding symptoms, including purpuras and ecchymoses of the lower extremities. Patients with factor V inhibitors and active bleeding can be treated with fresh frozen plasma [1], platelet concentrates [12], prothrombin complex concentrate, activated prothrombin complex concentrate, plasmapheresis, or immunoadsorption [1]. For the elimination of factor V inhibitors, possible provoking medications should be discontinued.…”