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

We report a case of acquired factor V inhibitors (AFVIs) in a patient with end-stage renal disease receiving warfarin therapy for atrial fibrillation. A 72-year-old Japanese man was admitted to our hospital complaining of tarry stools and abdominal pain. The laboratory findings revealed eosinophilia (52.1%), prolonged activated partial thromboplastin time (APTT) (98 s), PT (84 s), a factor V (FV) activity of <3%, and an FV inhibitor level of 6 Bethesda units/mL. After administration of prednisolone was started, his coagulation findings improved. However, his renal failure progressed, and he ultimately required chronic hemodialysis. This is the first case of AFVIs in a patient starting hemodialysis for end-stage renal disease.

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“…Some reports suggest that rituximab is effective in patients presenting with severely bleeding diathesis (13-16). …”

Section: Discussionmentioning

confidence: 99%

Acquired Factor V Inhibitors in a Patient with End-stage Renal Disease

et al. 2016

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“…Immunosuppressive regimens with corticosteroids alone or in association with cyclophosphamide or other immunosuppressants have been used successfully to suppress autoantibody production in 49 of 78 cases (63%), with remissions in 37 cases (76%). Among immunosuppressive agents, the anti-CD20 monoclonal antibody rituximab has been used successfully in three patients with severe and symptomatic acquired FV deficiency [74,77,84] with response in two cases. As for the management of acquired hemophilia A, this agent should be considered in those symptomatic patients resistant to standard immunosuppressive therapy.…”

Section: Search Methodsmentioning

confidence: 98%

“…It is difficult however to establish whether these latter conditions play a causative role in the development of the inhibitor because concomitant antibiotic therapy was also present in almost all cases. Interestingly, four acquired inhibitor cases [51,63,74,81] occurred after solid (3 liver transplants) or haematological (1 hematopoietic stem cell transplantation) transplants. A possible explanation for this phenomenon could be the posttransplant immune system dysregulation or, alternatively, the associated prophylactic use of antibiotics, so that coagulation monitoring (e.g., PT or APTT testing) might be advisable in patients undergoing these types of transplants.…”

Section: Search Methodsmentioning

confidence: 99%

Acquired factor V inhibitors: a systematic review

Franchini

Lippi

2010

J Thromb Thrombolysis

117

211

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The occurrence of an inhibitor against coagulation factor V (FV) is a rare but challenging condition, which may span from asymptomatic laboratory abnormalities to potentially life-threatening bleeding. The onset of FV inhibitors has been associated most frequently in the past with the patients' exposure to topical bovine thrombin administered during surgery procedures. However, since this preparation is no longer used, in this systematic review we have only focused on non-bovine thrombin-related FV inhibitor cases.

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“…Immunosuppressive regimens with corticosteroids and cyclophosphamide have been used successfully to suppress autoantibody production [56]. Finally, the anti-CD20 monoclonal antibody rituximab has also been used successfully in two patients with severe and symptomatic acquired factor V deficiency [57,58].…”

Section: Acquired Factor V Deficiencymentioning

confidence: 98%

Inherited and acquired factor V deficiency

Lippi

Favaloro

Montagnana

et al. 2011

Blood Coagulation &Amp; Fibrinolysis

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The clotting factor V, also known as proaccelerin or labile factor, is synthesized by the liver and possibly by the megakaryocytes. Factor V exerts a pivotal role in hemostasis, as it participates in both procoagulant and anticoagulant pathways, being an essential cofactor of the prothrombinase complex in the former case and participating in the inactivation of factor VIII (FVIII) in the latter. Isolated factor V deficiency due to mutations in the F5 gene is a rare inherited coagulopathy typically associated with a broad spectrum of bleeding symptoms, ranging from easy bruising, delayed bleeding after haemostatic challenges such as trauma or surgery to more severe joint bleeds. The combined deficiency of factor V and FVIII, commonly known as F5F8D, is a recessive disorder not attributable to the association of isolated factor V and FVIII deficiencies, but rather to defective intracellular processing of both proteins due to mutations involving the LMAN1 and MCFD2 genes, which encode two proteins forming an essential cargo receptor complex. Overall, patients affected by F5F8D do not bleed more in terms of both frequency and severity than those carrying specific deficiencies of both factors and the bleeding phenotype is generally mild. Although now increasingly rare, inhibitors directed against factor V may also develop in individuals of any age and are characterized by a very heterogeneous clinical phenotype. The aim of the current review is to provide an overview on the physiopathology, diagnostics, and clinical management of both inherited and acquired factor V deficiency.

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

Cited by 31 publications

References 14 publications

Acquired Factor V Inhibitors in a Patient with End-stage Renal Disease

Acquired Factor V Inhibitors in a Patient with End-stage Renal Disease

Acquired factor V inhibitors: a systematic review

Inherited and acquired factor V deficiency

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