Report of some cases of recurrent pregnancy loss due to congenital deficiency of factor XIII

Document Type : Case report


1 Assistant Professor, Department of Pediatrics, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Associate Professor, Department of Pediatrics, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


Introduction: Congenital deficiency of factor XIII is a kind of rare coagulating disorder which can cause recurrent pregnancy loss. The disease is manifested as spontaneous bleeding in the presence of normal coagulation tests. The women complicated with the disease in addition to heavy and prolonged menstrual bleeding are at risk of different pregnancy complications such as recurrent pregnancy loss. This study investigates recurrent abortion. This study introduces recurrent abortion in 2 sisters and tries to introduce this disease as a rare cause of recurrent pregnancy loss.
Case: This study introduces two sisters suffering from severe deficiency of factor XIII. The younger sister had referred after four abortions at the first trimester and the disease was diagnosed. After clotting factor replacement therapy, symptoms of disease were alleviated and successful pregnancy occurred after 4 months that led to the birth of a healthy term infant. The older sister was a 37 year old woman that the disease was diagnosed after 10 recurrent pregnancy losses. She was put through clotting factor replacement therapy, despite clotting factor replacement therapy and alleviation of hemorrhagic symptoms, pregnancy did not occur.
Conclusion: Accurate and on time diagnosis of factor XIII deficiency in women suffering from hemorrhagic disorders is necessary, and appropriate treatment can prevent of recurrent and sometimes serious hemorrhage, and recurrent pregnancy loss, and improve the quality of life.


  1. Sharief L, Kadir R. Congenital factor XIII deficiency in women: a systematic review of literature. Haemophilia 2013; 19(6):e349-57.
  2. Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII deficiency. Semin Semin Thromb Hemost 2009; 35(4):426-38.
  3. Kerlin B, Brand B, Inbal A, Halimeh S, Nugent D, Lundblad M, et al. Pharmacokinetics of recombinant factor XIII at steady state in patients with congenital factor XIII A‐subunit deficiency. J Thromb Haemost 2014; 12(12):2038-43.
  4. Asahina T, Kobayashi T, Takeuchi K, Kanayama N. Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature. Obstet Gynecol Surv 2007; 62(4):255-60
  5. 5. Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia 2008; 14(6):1190-200
  6. Bick RL, Hoppensteadt D. Recurrent miscarriage syndrome and infertility due to blood coagulation protein/platelet defects: a review and update. Clin Appl Thromb Hemost 2005; 11(1):1-13
  7. Pasquier E, De Saint Martin L, Kohler H, Schroeder V. Factor XIII plasma levels in women with unexplained recurrent pregnancy loss. J Thromb Haemost 2012; 10(4):723-5
  8. Naderi M, Eshghi P, Cohan N, Miri‐Moghaddam E, Yaghmaee M, Karimi M. Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran. Haemophilia 2012; 18(5):773-6
  9. Padmanabhan L, Mhaskar R, Mhaskar A, Ross C. Factor XIII deficiency: a rare cause of repeated abortions. Singapore Med J  2004; 45(4):186-7
  10. Inbal A, Muszbek L, editors. Coagulation factor deficiencies and pregnancy loss. Semin Thromb Hemost 2003; 29(2):171-4.
  11. Al-Sharif FZ, Aljurf MD, Al-Momen AM, Ajlan AM, Musa MO, Al-Nounou RM, et al. Clinical and laboratory features of congenital factor XIII deficiency. Saudi Med J 2002; 23(5):552-4.
  12. Dossenbach‐12. Glaninger A, Trotsenburg M, Oberkanins C, Atamaniuk J. Risk for Early Pregnancy Loss by Factor XIII Val34Leu: The Impact of Fibrinogen Concentration. J Clin Lab Anal  2013; 27(6):444-9.