Successful treatment with rituximab in a patient with an acquired factor V inhibitor

Lebrun, A; Leroy-Matheron, Catherine; Arlet, Jean‐Benoît; Bartolucci, Pablo; Michel, Marc

doi:10.1002/ajh.21056

Cited by 35 publications

(21 citation statements)

References 9 publications

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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: 99%

Acquired factor V inhibitors: a systematic review

Franchini

Lippi

2010

J Thromb Thrombolysis

115

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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Section: Search Methodsmentioning

confidence: 99%

Acquired factor V inhibitors: a systematic review

Franchini

Lippi

2010

J Thromb Thrombolysis

115

211

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“…Rituximab is an anti-CD20 monoclonal antibody that targets B lymphocytes and is used in the treatment of CD20-positive malignancies. Rituximab has also shown efficacy in multiple autoimmune disorders, including immune thrombocytopenia purpura, thrombotic thrombocytopenia purpura and acquired coagulation factor inhibitors [30]. In 2007, Raflores et al described the only case in the literature of successful use of plasmapheresis and steroids in combination with rituximab in a patient with HLAS [25].…”

Section: Treatmentmentioning

confidence: 99%

Resolution of Hypoprothrombinemia‐Lupus Anticoagulant Syndrome (HLAS) after multidrug therapy with rituximab: a case report and review of the literature

Paschal

Neff

2012

Haemophilia

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Hypoprothrombinemia associated with a lupus anticoagulant (LA) was first reported in the literature over 50 years ago. The hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare bleeding diathesis that has been associated with LAs in adult and paediatric patients with systemic lupus erythematosus (SLE) and with transient LAs due to other causes. There are no standard recommendations for treating haemorrhage associated with this syndrome. Herein, we report a patient with SLE and HLAS who achieved a durable remission following treatment with intravenous immune globulin (IVIG), prednisone and rituximab.

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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: 99%

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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Successful treatment with rituximab in a patient with an acquired factor V inhibitor

Cited by 35 publications

References 9 publications

Acquired factor V inhibitors: a systematic review

Acquired factor V inhibitors: a systematic review

Resolution of Hypoprothrombinemia‐Lupus Anticoagulant Syndrome (HLAS) after multidrug therapy with rituximab: a case report and review of the literature

Inherited and acquired factor V deficiency

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