Recommendations and suggestions for the treatment of women affected with RCDs
| Deficiency . | Notes . | |
|---|---|---|
| Afibrinogenemia | High level of fibrinogen is recommended to prevent early fetal loss (>1.0 g/L throughout pregnancy) and placental abruption during labor and to prevent PPH (ideally >2.0 g/L) | In women with a history of thrombotic episodes or other risk factors for venous thrombosis, postpartum management should take into account prophylaxis with low-molecular-weight heparin |
| Hypofibrinogenemia | Intrapartum replacement is required if the fibrinogen level is <1.5 mg/dL and/or the woman has a significant bleeding history; thrombosis events were reported during the puerperium, hence postpartum management should take into account any personal and family history of bleeding and thrombosis | |
| Dysfibrinogenemia | Dysfibrinogenemic women are at risk of both postpartum thrombosis and PPH; postpartum management of these women should be individualized based on their fibrinogen level as well as personal and family history of bleeding and thrombosis; women without symptoms should not be treated; data on genetic mutation and family history should also be taken into account | |
| FII | Limited available data | |
| FV | Women with low FV levels appear to be at increased risk of PPH, hence replacement therapy with FFP is recommended to raise FV level to >15%-25% | |
| FV + FVIII | Not enough information on pregnancy in these women; the obstetric experience of women with FV deficiency and carriers of hemophilia could probably serve as a useful guide in these patients: during labor FV levels should be >15% and FVIII levels >50% | |
| FVII | Women with low FVII levels (<10%-20%) or positive bleeding history are more likely to be at risk of PPH, therefore, prophylactic treatment should be considered | |
| FX | Patients with severe FX deficiency tend to be the most seriously affected patients with RCDs, therefore, they may benefit from replacement therapy during pregnancy and to cover labor and delivery to minimize the risk of bleeding complications; product containing FX to maintain FX level >30%-40% should be used; a pure specific FX is preferable | |
| FXI | The lack of correlation between FXI level and bleeding risk makes the management of FXI deficiency in pregnancy difficult; antifibrinolytic agents are effective for the majority of women with FXI deficiency, but those with severe deficiency may require FXI concentrate; rFVIIa has also been used to prevent bleeding, however, all treatments should be used with caution in pregnancy due to thrombogenic potential; the risk of PPH can be minimized by obstetric measures | |
| FXIII | Successful pregnancy in women with FXIII subunit A deficiency is only achieved with prophylaxis during pregnancy; target FXIII level should be >10%-20% until the week 22 of gestation; at the onset of labor, FXIII level need to be increased to >30% by shortening the therapeutic intervals | |
| Vitamin K dependent | 15 mg daily of oral vitamin K and administration of FFP in case of episitomy | |
| Deficiency . | Notes . | |
|---|---|---|
| Afibrinogenemia | High level of fibrinogen is recommended to prevent early fetal loss (>1.0 g/L throughout pregnancy) and placental abruption during labor and to prevent PPH (ideally >2.0 g/L) | In women with a history of thrombotic episodes or other risk factors for venous thrombosis, postpartum management should take into account prophylaxis with low-molecular-weight heparin |
| Hypofibrinogenemia | Intrapartum replacement is required if the fibrinogen level is <1.5 mg/dL and/or the woman has a significant bleeding history; thrombosis events were reported during the puerperium, hence postpartum management should take into account any personal and family history of bleeding and thrombosis | |
| Dysfibrinogenemia | Dysfibrinogenemic women are at risk of both postpartum thrombosis and PPH; postpartum management of these women should be individualized based on their fibrinogen level as well as personal and family history of bleeding and thrombosis; women without symptoms should not be treated; data on genetic mutation and family history should also be taken into account | |
| FII | Limited available data | |
| FV | Women with low FV levels appear to be at increased risk of PPH, hence replacement therapy with FFP is recommended to raise FV level to >15%-25% | |
| FV + FVIII | Not enough information on pregnancy in these women; the obstetric experience of women with FV deficiency and carriers of hemophilia could probably serve as a useful guide in these patients: during labor FV levels should be >15% and FVIII levels >50% | |
| FVII | Women with low FVII levels (<10%-20%) or positive bleeding history are more likely to be at risk of PPH, therefore, prophylactic treatment should be considered | |
| FX | Patients with severe FX deficiency tend to be the most seriously affected patients with RCDs, therefore, they may benefit from replacement therapy during pregnancy and to cover labor and delivery to minimize the risk of bleeding complications; product containing FX to maintain FX level >30%-40% should be used; a pure specific FX is preferable | |
| FXI | The lack of correlation between FXI level and bleeding risk makes the management of FXI deficiency in pregnancy difficult; antifibrinolytic agents are effective for the majority of women with FXI deficiency, but those with severe deficiency may require FXI concentrate; rFVIIa has also been used to prevent bleeding, however, all treatments should be used with caution in pregnancy due to thrombogenic potential; the risk of PPH can be minimized by obstetric measures | |
| FXIII | Successful pregnancy in women with FXIII subunit A deficiency is only achieved with prophylaxis during pregnancy; target FXIII level should be >10%-20% until the week 22 of gestation; at the onset of labor, FXIII level need to be increased to >30% by shortening the therapeutic intervals | |
| Vitamin K dependent | 15 mg daily of oral vitamin K and administration of FFP in case of episitomy | |
PPH, postpartum hemorrhage.