Near fatal spontaneous intraperitoneal bleeding: A rare manifestation in a congenital factor X deficiency carrier

Vinod, Kolar Vishwanath; Babu, Hitha; Kaaviya, Ramesh; Tk, Dutta

doi:10.4103/0972-5229.152774

Cited by 4 publications

(6 citation statements)

References 8 publications

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“…In total, 332 women were identified; 30 women in 30 case reports (Table ) and 302 women in 19 case series. In the case reports, the FX coagulant activity was <0.01 IU/mL in 14 women, 0.01‐0.05 IU/mL in 8 women and >0.05 IU/mL in four women, and in four women, the severity of FX deficiency was not mentioned . In the case series, 63 patients had FX coagulant activity ≤0.01 IU/mL, 95 patients >0.01 IU/mL (range 0.01‐0.26) and in the rest FX level was not reported.…”

Section: Resultsmentioning

confidence: 93%

“…Haemoperitoneum from ovulation bleeding/ruptured haemorrhagic cyst occurred in 8 (2.4%) women (Table ). All cases required blood transfusion.…”

Section: Resultsmentioning

confidence: 99%

“…All cases required blood transfusion. FFP was used in six women . Surgical management (laparoscopy) was required in six (75%) cases and conversion to laparotomy in four women .…”

Section: Resultsmentioning

confidence: 99%

“…43 In 18 cases, the treatment prescribed was not mentioned. 40,41,46 Haemoperitoneum from ovulation bleeding/ruptured haemorrhagic cyst occurred in 8 (2.4%) women 13,15,16,18,25,31,36,37 ( Table 2). All cases required blood transfusion.…”

Section: Re Sultsmentioning

confidence: 99%

“…FFP was used in six women. 15,16,18,25,31,37 Surgical management (laparoscopy) was required in six (75%) cases 13,15,18,25,31,36 and conversion to laparotomy in four women. 18,25,36 Four women had ovarian cystectomy, 15,16,25,37 and two (25%) had oophorectomy.…”

Section: Re Sultsmentioning

confidence: 99%

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Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. A total number of 49 relevant articles were identified, including 332 women, dating from 1960 to 2018. Heavy menstrual bleeding was reported in 72/284 (25%) women in total, 14/30 (47%) in case reports and 58/254 (23%) in 11 case series, 64% and 10% required blood products and blood transfusion, respectively. Haemoperitoneum from ovulation bleeding or ruptured haemorrhagic ovarian cyst requiring blood transfusion occurred in 8/322 (2.4%) women, six required surgical intervention, including oophorectomy in two. 31 pregnancies were reported in 19 women. There were four miscarriages (including a late miscarriage at 21 weeks). There was a high rate of preterm birth and neonatal death occurring in eight (30%) and three (11%) of pregnancies reaching viability stage. Postpartum haemorrhage (PPH) occurred in six (22%) of deliveries, one requiring hysterectomy. In conclusion, women with FXD are at an increased risk of heavy bleeding during menstruation and ovulation as well as adverse pregnancy outcome and postpartum haemorrhage. Collaboration in a multidisciplinary team including an obstetrician/gynaecologist, a perinatologist and a haematologist is necessary for the prevention and management of these complications.

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Section: Resultsmentioning

confidence: 93%

“…Haemoperitoneum from ovulation bleeding/ruptured haemorrhagic cyst occurred in 8 (2.4%) women (Table ). All cases required blood transfusion.…”

Section: Resultsmentioning

confidence: 99%

“…All cases required blood transfusion. FFP was used in six women . Surgical management (laparoscopy) was required in six (75%) cases and conversion to laparotomy in four women .…”

Section: Resultsmentioning

confidence: 99%

Section: Re Sultsmentioning

confidence: 99%

Section: Re Sultsmentioning

confidence: 99%

See 3 more Smart Citations

Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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Factor IX(a) inhibitors: an updated patent review (2003-present)

Afosah

Ofori

Mottamal

et al. 2022

Expert Opinion on Therapeutic Patents

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A novel factor X mutation Cys81 by Arg and a reported factor VII polymorphism Arg353 replaced by Gln co-occured in a patient

Jin

Cheng

Zheng

et al. 2018

Blood Coagulation &Amp; Fibrinolysis

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: Coagulation factor X and factor VII (FVII) are both very important components in blood coagulation. To study the molecular pathogenic mechanism of the inherited factor X and FVII deficiency, the factor X activity (FX:C) and FVII activity were tested with one-stage clotting methods. The factor X antigen and factor FVII antigen were tested with ELISA. All the exons, intron-exon boundaries and 5',3'-flanking regions of F10 and F7 genes were amplified by PCR with direct sequencing. The ClustalX software was used to analyze the conservative property of the mutation sites. The PolyPhen-2 and Sorting Intolerant From Tolerant (SIFT) online bioinformatics softwares were taken to predict whether the point mutation could affect protein function. The software Swiss-pdb Viewer was brought to analyze the impact of mutations on the structure and function of the protein. The thrombin generation tests were applied to evaluate whether there were obstacles in producing thrombin about the mutant protein. The FX:C and FVII activity of the proband were reduced to 35 and 42%, and the factor X antigen and factor FVII antigen were decreased to 43 and 55%, simultaneously. Correspondingly, a FX:Cys81Arg (Cys81 by Arg) mutation and a FVII:Arg353 replaced by Gln polymorphism were detected in the proband. The Cys81 of factor X was conserved among homologous species, but the Arg353 of FVII was not. All softwares analysis results indicated protein features and structures might be affected by the mutation and the polymorphism. And the thrombin generation tests showed that the mutant protein had obstacles in thrombin generation. We identified a FX:Cys81Arg mutation and a FVII:Arg353 replaced by Gln polymorphism in the proband. And they accounted for the decrease of the activity and antigen of factor X and FVII. Of note, the Cys81Arg of factor X was first reported in the world.

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Near fatal spontaneous intraperitoneal bleeding: A rare manifestation in a congenital factor X deficiency carrier

Cited by 4 publications

References 8 publications

Congenital Factor X deficiency in women: A systematic review of the literature

Congenital Factor X deficiency in women: A systematic review of the literature

Factor IX(a) inhibitors: an updated patent review (2003-present)

A novel factor X mutation Cys81 by Arg and a reported factor VII polymorphism Arg353 replaced by Gln co-occured in a patient

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