Advertisement
Review Article| Volume 35, ISSUE 6, P1171-1180, December 2021

Download started.

Ok

Factor XIII Deficiency

A Review of Clinical Presentation and Management
  • Ari Pelcovits
    Affiliations
    Alpert Medical School of Brown University, Providence, RI, USA

    Division of Hematology-Oncology, Rhode Island Hospital and The Miriam Hospital, 164 Summit Avenue, Providence, RI 02906, USA
    Search for articles by this author
  • Fred Schiffman
    Affiliations
    Alpert Medical School of Brown University, Providence, RI, USA

    Division of Hematology-Oncology, Rhode Island Hospital and The Miriam Hospital, 164 Summit Avenue, Providence, RI 02906, USA
    Search for articles by this author
  • Rabin Niroula
    Correspondence
    Corresponding author. Warren Alpert Medical School of Brown University, Rhode Island Hospital, 593 Eddy Street, Providence, RI 02903.
    Affiliations
    Alpert Medical School of Brown University, Providence, RI, USA

    Division of Hematology-Oncology, Rhode Island Hospital and The Miriam Hospital, 164 Summit Avenue, Providence, RI 02906, USA
    Search for articles by this author
Published:October 01, 2021DOI:https://doi.org/10.1016/j.hoc.2021.07.009

      Keywords

      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'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Hematology/Oncology Clinics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Dorgalaleh A.
        • Naderi M.
        • Shamsizadeh M.
        Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency.
        Ann Hematol. 2016; 95: 451-455
        • Sharief L.A.
        • Kadir R.A.
        Congenital factor XIII deficiency in women: a systematic review of literature.
        Haemophilia. 2013; 19: e349-e357
        • Hsieh L.
        • Nugent D.
        Factor XIII deficiency.
        Haemophilia. 2008; 14: 1190-1200
        • Pisano J.J.F.J.
        • Peyton M.P.
        Cross-link in fibrin polymerized by factor 13: epsilon-(gamma-glutamyl)lysine.
        Science. 1968; 160: 892-893
        • Naderi M.
        • Dorgalaleh A.
        • Alizadeh S.
        • et al.
        Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency.
        Int J Hematol. 2014; 100: 443-449
        • Palla R.
        • Shapiro A.D.
        Rare bleeding disorders: diagnosis and treatment.
        Blood. 2015; 125: 2052-2061
        • Anwar R.
        Factor XIII deficiency.
        Br J Haematol. 1999; 107: 468-484
        • Bouttefroy S.
        • Meunier S.
        • Milien V.
        • et al.
        Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort.
        Br J Haematol. 2020; 188: 317-320
        • Koseki-Kuno S.
        • Yamakawa M.
        • Dickneite G.
        • et al.
        Factor XIII a subunit-deficient mice developed severe uterine bleeding events and subsequent spontaneous miscarriages.
        Blood. 2003; 102: 4410-4412
        • Asahina T.K.T.
        • Takeuchi K.
        • Kanayama N.
        Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature.
        Obstet Gynecol Surv. 2007; 62: 255-260
        • Peyvandi F.
        • Palla R.
        • Menegatti M.
        • et al.
        Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders.
        J Thromb Haemost. 2012; 10: 615-621
        • Menegatti M.
        • Palla R.
        • Boscarino M.
        • et al.
        Minimal factor XIII activity level to prevent major spontaneous bleeds.
        J Thromb Haemost. 2017; 15: 1728-1736
        • Yan M.T.S.
        • Rydz N.
        • Goodyear D.
        • et al.
        Acquired factor XIII deficiency: a review.
        Transfus Apher Sci. 2018; 57: 724-730
        • Nixon C.P.P.E.
        • Guertin C.A.
        • Stevenson R.L.
        • et al.
        Acquired Factor XIII inhibitor associated with mantle cell lymphoma.
        Transfusion. 2017; 57: 694-699
        • Ichinose A.
        • Japanese Collaborative Research Group on A.H.
        Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: a summary of 93 patients.
        Blood Rev. 2017; 31: 37-45
        • Kohler H.P.
        • Seitz R.
        • Ariens R.A.
        • et al.
        Factor XIII and fibrinogen SSC subcommittee of the ISTH. Diagnosis and classification of factor XIII deficiencies.
        J Thromb Haemost. 2011; 9: 1404-1406
        • Mannucci P.M.
        • Duga S.
        • Peyvandi F.
        Recessively inherited coagulation disorders.
        Blood. 2004; 104: 1243-1252
        • Winkelman L.
        • Haddon M.E.
        • Evans D.R.
        • et al.
        A pasteurized concentrate of human plasma factor XIII for therapeutic use.
        Thromb Haemost. 1986; 55: 402-405
        • Nugent D.
        Corifact™/Fibrogammin® P in the prophylactic treatment of hereditary factor XIII deficiency: results of a prospective, multicenter, open-label study.
        Thromb Res. 2012; 130: S12-S14
        • Solomon C.
        • Fries D.
        • Pendrak I.
        • et al.
        Safety of factor XIII concentrate: analysis of more than 20 years of pharmacovigilance data.
        Transfus Med Hemother. 2016; 43: 365-373
        • Inbal A.
        • Oldenburg J.
        • Carcao M.
        • et al.
        Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency.
        Blood. 2012; 119: 5111-5117
        • Bolton-Maggs P.H.
        • Perry D.J.
        • Chalmers E.A.
        • et al.
        The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation.
        Haemophilia. 2004; 10: 593-628
        • Jennings I.
        • Woods T.A.
        • Preston F.E.
        • et al.
        Problems relating to the laboratory diagnosis of factor XIII deficiency: a UK NEQAS study.
        J Thromb Haemost. 2003; 1: 2603-2608
        • Asahina T.
        • Kobayashi T.
        • Okada Y.
        • et al.
        Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell.
        Placenta. 2000; 21: 388-393
        • Janbain M.
        • Nugent D.J.
        • Powell J.S.
        • et al.
        Use of factor XIII (FXIII) concentrate in patients with congenital FXIII deficiency undergoing surgical procedures.
        Transfusion. 2014; 55: 45-50
        • Carcao M.
        • Altisent C.
        • Castaman G.
        • et al.
        Recombinant FXIII (rFXIII-A2) prophylaxis prevents bleeding and allows for surgery in patients with congenital FXIII A-subunit deficiency.
        Thromb Haemost. 2018; 118: 451-460
        • Lak M.
        • Ali Sharifian A.
        • Karimi K.
        • et al.
        Pattern of symptoms in 93 Iranian patients with severe factor XIII deficiency.
        J Thromb Haemost. 2003; 1: 1852-1853
        • Acharya S.S.
        • Dimichele D.M.
        Rare bleeding disorder registry: de®ciencies of factors II, V, VII,X, XIII, ®brinogen and dys®brinogenemias.
        J Thromb Haemost. 2004; 2: 248-256
        • Ivaskevicius V.
        • Kohler H.P.
        • Schroeder V.
        • et al.
        • Study Group
        International registry on factor XIII deficiency: a basis formed mostly on European data.
        Thromb Haemost. 2007; 97: 914-921
        • Viswabandya A.
        • Baidya S.
        • Nair S.C.
        • et al.
        Correlating clinical manifestations with factor levels in rare bleeding disorders: a report from Southern India.
        Haemophilia. 2012; 18: e195-e200