Sifa Medical Journal

CASE REPORT
Year
: 2014  |  Volume : 1  |  Issue : 2  |  Page : 21--23

Cesarean section and ovarian cystectomy in the patient with factor XI deficiency


Guluzar Arzu Turan1, Melike Yuksel Yavuz1, Esra Bahar Gur1, Sumeyra Tatar1, Irem Hepyilmaz1, Ulku Ergene2,  
1 Department of Obstetrics and Gynecology, Sifa University Hospital, Izmir, Turkey
2 Department of Hematology, Sifa University Hospital, Izmir, Turkey

Correspondence Address:
Guluzar Arzu Turan
Sanayi Cad No. 7 Bornova, Izmir 35100
Turkey

Abstract

Factor XI (F XI) deficiency is a rare bleeding disorder with an autosomal inheritance and generally asymptomatic. However, the incidence of primary post-partum hemorrhage and secondary post-partum hemorrhage in F XI deficiency were reported as 16% and 24%, respectively. A 35-year-old primigravida woman diagnosed with F XI deficiency was examined by the maternal-fetal medicine service of our hospital at 37 weeks 5 days«SQ» gestation. Her blood levels of F XI and activated partial thromboplastin time were %2 and 116 s, respectively at pre-partum period. We made fresh frozen plasma (FFP) replacement 1 day before surgery and we continued transfusions until post-operative 6 th day. The most challenging problems we encountered were hypervolemia and electrolyte imbalance. The management of F XI deficiency necessitates careful consideration and replacement with FFP is believed to be the main approach.



How to cite this article:
Turan GA, Yavuz MY, Gur EB, Tatar S, Hepyilmaz I, Ergene U. Cesarean section and ovarian cystectomy in the patient with factor XI deficiency.Sifa Med J 2014;1:21-23


How to cite this URL:
Turan GA, Yavuz MY, Gur EB, Tatar S, Hepyilmaz I, Ergene U. Cesarean section and ovarian cystectomy in the patient with factor XI deficiency. Sifa Med J [serial online] 2014 [cited 2021 Sep 21 ];1:21-23
Available from: https://www.imjsu.org/text.asp?2014/1/2/21/132964


Full Text

 Introduction



Factor XI (F XI) deficiency is a rare bleeding disorder with an autosomal inheritance. [1] It was first described in 1953 in a Jewish family. [2] F XI deficiency is generally asymptomatic and spontaneous bleeding is uncommon. Whereas delayed post-operative bleeding and bleeding after injury are often the presentation. The severity of the bleeding is not predictable and does not relate to the F XI level. [3] The incidence of primary post-partum hemorrhage (PPPH) and secondary post-partum hemorrhage (SPPH) in general obstetric population are 5% and 0.7%, respectively. [4] However, the incidence of PPPH and SPPH in F XI deficiency were reported as 16% and 24%, respectively. [4] We aimed to emphasize that the proper management of a pregnant with F XI deficiency and control of F XI levels are crucial during third trimester as F XI deficiency shows clinical variabilities in pregnancy and there is no chance to measure F XI level at emergent states.

 Case Report



The present case is about a 35-year-old primigravida woman was seen by the maternal-fetal medicine service of our hospital at 37 weeks 5 days' gestation because she has been diagnosed with F XI deficiency when she had severe bleeding after dental extraction. Her blood levels of F XI and activated partial thromboplastin time (APTT) were %2 and 116 s, respectively at pre-partum period. We made fresh frozen plasma (FFP) replacement 1 day before surgery and we continued transfusions until post-operative 6 th day to reduce the possibility of delayed post-operative bleeding. Under general anesthesia, cesarean section was performed firstly and cystectomy was done as we observed an 8 cm × 10 cm left ovarian cystic mass including some fat, cartilage and bone tissue, two teeth and clumps of hair compatible with dermoid cyst pre-operatively [Figure 1]. Her APTT levels could be controlled by transfusions with 74 units of FFP from the 1 st pre-operative day to post-operative 6 th day [Table 1] and [Table 2]. There was not bleeding from surgical incision and the amount of vaginal bleeding was three pad lochia/day. D-dimer levels increased on post-operative 2 nd day and decreased to normal levels without intervention. The most challenging problems we encountered were hypervolemia and electrolyte imbalance. The patient was discharged from hospital at post-operative 7 th day with stable clinical findings.{Table 1}{Table 2}{Figure 1}

 Discussion



F XI is a fibrinolysis inhibitor and fibrin stabilator. The replacement treatment with FFP is generally necessary if F XI values are <15%. [5] Myers et al. have reported that 70% of pregnant women had stable pregnancy and labor in their study including 33 pregnant women with F XI deficiency which is consistent with the study that Salomon et al. showed the rate of women did not experience PPH as 69.4% (43 of 62 women). They also found that the rate of PPH is increased in symptomatic patient group. [6],[7] In another study, the rate of PPH in F XI deficiency was estimated as 24%. [4] For surgery, general anesthesia is the first choice preferably. [8] The management of F XI deficiency necessitates careful consideration and replacement with FFP is believed to be the main approach, whereas delayed post-operative bleeding is often the presentation of F XI deficiency. [9] We should be careful about hypervolemia, viral transmission, immunologic reactions such as allergy/anaphylaxis, transfusion-related acute lung injury and hemolysis. Plasma-derived F XI concentrates may become alternative treatments but cardiac complications and pulmonary embolism due to thrombotic effects are reported. Low-dose recombinant activated factor VIIa, cryoprecipitates and desmopressin may become alternative treatment options. [3],[9],[10] We also preferred replacement with FFP transfusion as a treatment approach.

References

1Seligsohn U. Factor XI deficiency. Thromb Haemost 1993;70:68-71.
2Rosenthal RL, Dreskin OH, Rosenthal N. New hemophilia-like disease caused by deficiency of a third plasma thromboplastin factor. Proc Soc Exp Biol Med 1953;82:171-4.
3Bolton-Maggs PH. Factor XI deficiency - Resolving the enigma? Hematology Am Soc Hematol Educ Program 2009;1:97-105.
4Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. Pregnancy in women with von Willebrand's disease or factor XI deficiency. Br J Obstet Gynaecol 1998;105:314-21.
5Chauleur C, Cochery-Nouvellon E, Mercier E, Aya G, Fabbro-Peray P, Mismetti P, et al. Some hemostasis variables at the end of the population distributions are risk factors for severe postpartum hemorrhages. J Thromb Haemost 2008;6:2067-74.
6Myers B, Pavord S, Kean L, Hill M, Dolan G. Pregnancy outcome in Factor XI deficiency: Incidence of miscarriage, antenatal and postnatal haemorrhage in 33 women with Factor XI deficiency. BJOG 2007;114:643-6.
7Salomon O, Steinberg DM, Tamarin I, Zivelin A, Seligsohn U. Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency. Blood Coagul Fibrinolysis 2005;16:37-41.
8Singh A, Harnett MJ, Connors JM, Camann WR. Factor XI deficiency and obstetrical anesthesia. Anesth Analg 2009;108:1882-5.
9Steward RG, Saleh OA, James AH, Shah AA, Price TM. Management of gynecologic surgery in the patient with factor XI deficiency: A review of the literature. Obstet Gynecol Surv 2012;67:291-7.
10Kadir RA, Economides DL, Lee CA. Factor XI deficiency in women. Am J Hematol 1999;60:48-54.