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Factor XIII deficiency management: a review of the literature

Odame, Jodie E.a; Chan, Anthony K.b,c; Wu, John K.d,e; Breakey, Vicky R.b,c

Blood Coagulation & Fibrinolysis: April 2014 - Volume 25 - Issue 3 - p 199–205
doi: 10.1097/MBC.0000000000000029

Factor XIII (FXIII) deficiency is a rare congenital bleeding disorder estimated to affect 1 in 2 million live births. Treatment often involves prophylaxis with FXIII concentrate and is especially important in preventing intracranial hemorrhage (ICH) and maintaining pregnancy in women of childbearing age. The rarity of this condition and lack of good quality evidence has resulted in a literature largely based on case reports/case series. A review of the literature was conducted in order to provide information about the optimal management of FXIII deficiency. Articles were identified by searching MEDLINE from 1961 to June 2012. Eligible studies included details on patients with FXIII deficiency that received treatment. Information collected included dose, frequency, duration, hemostatic efficacy and adverse events. Of 606 abstracts reviewed, 43 articles, including a total of 328 patients met the selection criteria. Common bleeding manifestations included umbilical cord bleeding, ICH and hematomas. Patients were generally placed on prophylactic factor replacement therapy upon diagnosis of severe or symptomatic FXIII deficiency, which decreased and/or prevented bleeding episodes. Patients with FXIII deficiency that received prophylactic treatment successfully maintained pregnancies. Alternative treatments included the use of cryoprecipitate or frozen plasma when FXIII concentrate was not available or affordable. Recent studies of a new recombinant FXIII concentrate show promising results in regards to safety and efficacy. There are limited data to guide the optimal treatment of FXIII deficiency. Larger patient registries and international collaborations are needed to improve the evidence and enhance clinical outcomes in this rare bleeding disorder.

aDepartment of Physiology, University of Toronto, Toronto, Ontario

bDepartment of Pediatrics, McMaster University, Hamilton, Ontario

cDivision of Hematology/Oncology, McMaster Children's Hospital, Hamilton, Ontario

dDepartment of Pediatrics, University of British Columbia, Vancouver, British Columbia

eDivision of Pediatric Hematology/Oncology, British Columbia Children's Hospital, Vancouver, British Columbia, Canada

Correspondence to Vicky R. Breakey, MD, MEd, FRCPC, Pediatric Hematologist/Oncologist, Assistant Professor, Division of Pediatric Hematology/Oncology, McMaster Children's Hospital, HSC 3N27a – 1280 Main St. W, Hamilton, Ontario L8S 4K1, Canada Tel: +1 905 521 2100 x73080; fax: +1 905 521 1703; e-mail:

Received 3 April, 2013

Revised 22 October, 2013

Accepted 13 November, 2013

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins