Letter to the Editors-in-Chief| Volume 164, P1-3, April 2018

Download started.


Acquired factor V deficiency in a patient with myeloma and amyloidosis

Published:February 04, 2018DOI:


      • Acquired factor V deficiency (aFVd) is a rare bleeding disorder commonly caused by autoantibodies against factor V.
      • Isolated acquired factor V deficiency associated with plasma cell dyscrasia is rare.
      • In the absence of a demonstrable inhibitor, the mechanism of aFVD acquired could be either from adsorption of factor V by amyloid deposits or mediated by light chains that were significantly elevated.
      • Plasma cell dyscrasia should be sought as a cause for aFVD, in particular one where bleeding manifestation is profound.



      We describe our experience with managing an unusual case of acquired Factor V deficiency (aFVd) in a myeloma patient with demonstrated amyloidosis.


      Following diagnosis, records of previous investigations were sought. Specific clotting factors and inhibitors were tested. The clinical progress and treatment response measured by serial factor V levels and coagulation parameters was then prospectively tracked.


      A 57 year-old woman presented with spontaneous right knee haemarthrosis in association with bilateral symmetrical polyneuropathy and proteinuria. Coagulation screen showed prolongation of both PT (18.6 s, normal range [9.9–11.4 s]) and aPTT (41.4 s, normal range [25.7–32.9 s]), which were both fully correctable following a mixing study. Liver function, fibrinogen, clotting factor II/VIII/X assays and disseminated intravascular coagulopathy screen was normal. FV level was reduced (19%, normal range [70–170%]). Inhibitor titer was undetectable. Congenital FVd was excluded as her previous coagulation screen was normal. Bone marrow investigation performed for suspected underlying plasma cell dyscrasia showed 60% neoplastic plasma cells. Congo red staining was positive for amyloid within vascular walls of the marrow trephine. She was diagnosed with light chain myeloma and aFVd. She received Bortezomib/Cyclophosphamide/Dexamethasone (VCD) chemotherapy. After one cycle of VCD, serum kappa free light chain (SFLC) was reduced from 6951 mg/L to 3354 mg/L with serial measurements of FV levels showing increment to 76% and normalization of PT/aPTT.


      Plasma cell dyscrasia with amyloidosis should be sought as a cause for aFVD, in particular one where bleeding manifestation is profound even with the absence of demonstrable inhibitors.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Thrombosis Research
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Ang A.L.
        • Kuperan P.
        • Ng C.H.
        • Ng H.J.
        Acquired factor V inhibitor. A problem-based systematic review.
        Thromb. Haemost. 2009; 101: 852-859
        • Furie B.
        • Voo L.
        • McAdam K.P.
        • Furie B.C.
        Mechanism of factor X deficiency in systemic amyloidosis.
        N. Engl. J. Med. 1981; 304: 827-830
        • Harrison Jonathan S.
        • et al.
        Evidence of both von Willebrand factor deposition and factor V deposition onto AL amyloid as the cause of a severe bleeding diathesis.
        Blood Coagul. Fibrinolysis. 2017; 28: 342-347
        • Emori Y.
        • Sakugawa M.
        • Niiya K.
        • Kiguchi T.
        • Kojima K.
        • Takenaka K.
        • et al.
        Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis.
        Blood Coagul. Fibrinolysis. 2002; 13: 555-559
        • Gatel A.
        • Cacoub P.
        • Piette J.C.
        AL amyloidosis combined with acquired factor V deficiency.
        Ann. Intern. Med. 1998; 128: 604-605
        • Sucker C.
        • Hetzel G.R.
        • Grabensee B.
        • Stockschlaeder M.
        • Scharf R.E.
        Amyloidosis and bleeding: pathophysiology, diagnosis, and therapy.
        Am. J. Kidney Dis. 2006; 47: 947-955
        • Sosa I.R.
        • Ellery P.
        • Mast A.
        • Neff A.T.
        • Gailani D.
        Acquired factor V deficiency in a patient without evidence of a classical inhibitor.
        Haemophilia. 2014; 20
        • Choufani E.B.
        • Sanchorawala V.
        • Ernst T.
        • Quillen K.
        • Skinner M.
        • Wright D.G.
        • et al.
        Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy.
        Blood. 2001; 97: 1885-1887