Danazol therapy in factor X deficiency

Mukhopadhyay, Sanjay; Saxena, Renu; Kashyap, Rajesh; Choudhry, V. P.

doi:10.1046/j.1365-2516.2001.00541.x

Cited by 6 publications

(16 citation statements)

References 15 publications

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“…There is another purported case of FX deficiency with suspected venous thrombosis in the literature [25]. The patient is a 21 years old female with a FX activity of about 4% of normal who complained of bilateral calf pain.…”

Section: Discussionmentioning

confidence: 99%

The clinical significance of the lack of arterial or venous thrombosis in patients with congenital prothrombin or FX deficiency

Girolami

Candeo

Vettore

et al. 2009

J Thromb Thrombolysis

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Several reports have dealt with the occurrence of both arterial and venous thrombosis in patients with haemophilia A, haemophilia B, and von Willebrand disease. Similar thrombotic events have been occasionally reported also in rare congenital coagulation disorders, particularly in fibrinogen or FVII deficiencies. On the contrary no sure venous or arterial thrombotic event has ever been reported in congenital prothrombin or Factor X deficiency. The significance of this observation is discussed. This discrepancy cannot be explained on the basis of the rarity of the two conditions, since in similarly rare congenital bleeding disorders such as FV or FXIII deficiency a few patients with thrombosis have been described. It appears that only these two defects are able to allow a sure protection from thrombosis. These observations may indirectly support the rationale for the use of direct thrombin or Factor X inhibitors in the prophylaxis and/or therapy of thrombotic manifestations.

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Section: Discussionmentioning

confidence: 99%

The clinical significance of the lack of arterial or venous thrombosis in patients with congenital prothrombin or FX deficiency

Girolami

Candeo

Vettore

et al. 2009

J Thromb Thrombolysis

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“…Prophylaxis products, regimens and efficacy are well-established in severe haemophilia A and B but in severe FX deficiency, despite extensive reviews of the disease [1], with fewer data and no purified factor these issues are less clear. There is one report of successful long-term prophylaxis using FFP in a severely FX deficient infant with recurrent ICH [6] and regular Danazol has also been tried although with variable results [7]. However, most reports of long-term prophylaxis in FX deficiency concern PCCs [8][9][10][11][12][13][14][15][16].…”

Section: Discussionmentioning

confidence: 99%

Severe factor X deficiency due to a homozygous mutation (Cys364Arg) that disrupts a disulphide bond in the catalytic domain

et al. 2006

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Severe factor X deficiency (<0.01 IU mL(-1)) is a rare disorder producing a major bleeding tendency including umbilical cord, joint and intracranial haemorrhage. We present the first case of a child homozygous for a g.1177T > C missense alteration, predicted to disrupt the catalytic domain, and resulting in severe FX deficiency. The child suffered intracranial haemorrhage and now receives regular prophylaxis with a prothrombin complex concentrate. Our experience and a review of the literature suggest that optimal frequency of dosing is likely to be two or three times weekly and that the risk of thrombosis is very small.

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“…48 Progestins were used in three cases 38,48 and danazol in another one. 23 One woman had a hysterectomy. 43 In 18 cases, the treatment prescribed was not mentioned.…”

Section: Re Sultsmentioning

confidence: 99%

“…Flow diagram of literature review TA B L E 2 Obstetric and gynaecological bleeding episodes in women with FX deficiency severity of FX deficiency was not mentioned 3,[32][33][34]. 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.Heavy menstrual bleeding was a common bleeding symptom reported in 72/284 (25%) in total, 14/30 (47%) in case reports,3,7,9,14,15,17,19,22,23,[25][26][27][35][36][37] and 58/254 (23%) in 11 case series38,39 (Table 2). Semi-objective assessment of HMB in terms of pictorial blood assessment chart was not reported.…”

mentioning

confidence: 99%

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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Danazol therapy in factor X deficiency

Cited by 6 publications

References 15 publications

The clinical significance of the lack of arterial or venous thrombosis in patients with congenital prothrombin or FX deficiency

The clinical significance of the lack of arterial or venous thrombosis in patients with congenital prothrombin or FX deficiency

Severe factor X deficiency due to a homozygous mutation (Cys364Arg) that disrupts a disulphide bond in the catalytic domain

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

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