|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 3 | Page : 225-227
Management of von willebrand disease in pregnancy
Shiny Varghese, Divya Mahindru
Department of Obstetrics and Gynaecology, Christian Medical College and Hospital, Ludhiana, Punjab, India
|Date of Web Publication||13-Jul-2017|
Department of Obstetrics and Gynaecology, Christian Medical College and Hospital, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Varghese S, Mahindru D. Management of von willebrand disease in pregnancy. CHRISMED J Health Res 2017;4:225-7
| Introduction|| |
Von Willebrand disease (VWD) is the most common inherited bleeding coagulopathy  resulting from deficiency or defect in von Willebrand factor (VWF), the large multimeric protein which mediates platelet adhesion and serves as a carrier protein for factor VIII (FVIII). Three types of VWD have been described: type 1, type 2, and type 3. Type 1 is the result of a partial, quantitative deficiency of a structurally normal VWF, and accounts for 70%–80% of all VWD patients. Type 2 (20% of VWD patients) includes several qualitative defects in VWF that affect its multimeric structure or function. Type 3 (5%–10% of VWD patients) is the most severe form of the disease resulting from a complete deficiency of VWF and a secondary severe deficiency of FVIII.
During normal pregnancy, there is a progressive increase in FVIII and VWF levels. Most studies suggest an increase beginning during the second trimester, with peak levels at term, followed by a return to baseline during the postpartum period. Ideally, patients are treated with platelet transfusion, however, the majority require treatment with a plasma–derived VWF/FVIII concentrates and cryoprecipitate. Partial correction of plasma VWF level, however, has been effective in some patients as reviewed by Miller et al. An individualized approach is suggested for both treatment and prophylaxis in VWD.
| Case Report|| |
A 32-year-old G2P0+1 presented to the antenatal clinic at 35 weeks gestation with a known diagnosis of VWD type 2. The patient had been asymptomatic with this condition until diagnosis in childhood following epistaxis. The disease runs in her family with her siblings being affected with the similar condition. She received multiple blood transfusions since childhood in view of her falling hemoglobin. Her pubertal period showed menorrhagia which was managed conservatively with tranexamic acid and hormonal therapy. At the time of her antenatal booking, she was asymptomatic and was not undergoing any treatment for her condition. A multi-disciplinary team (MDT) was involved in her antenatal, intrapartum and postpartum care which along with an obstetrician included hematologists, anesthetists, and neonatologists.
The antenatal plan was discussed with the patient during several visits. Her routine antenatal investigations were done. Serial ultrasonograms for fetal well-being were done which showed normal fetal growth with no placental abnormalities. She was diagnosed to have intrahepatic cholestasis of pregnancy at 35–36 weeks of gestation. Hence, a plan was made for induction at around 38 weeks of gestation. To facilitate a safe pregnancy and delivery, the following care plan was made:
- Regular follow-ups with obstetrician and hematologists
- Regular hemoglobin and platelet counts
- VWF assay and ristocetin assays
- Blood coagulation profile
The VWF and ristocetin assays were deranged suggesting VWD disease with deranged coagulation (activated partial thromboplastin time).
She was admitted at 38 weeks of gestation for induction of labor as planned.
- Hematologists and anesthesiologists were informed
- Blood and blood products (cryoprecipitates) were arranged in accordance with hematologist's advice
- VWF and FVIII were arranged for
- Epidural (regional analgesia) was contraindicated as suggested by hematology team
- During labor, pain relief was offered in the form of injection tramadol as an intravenous infusion.
She was taken up for the emergency cesarean section in view of cephalopelvic disproportion. Before shifting her for lower segment cesarean section, the antihemophilic factor/VWF complex (human) dried, pasteurized-Humate-P was injected [Figure 1],[Figure 2],[Figure 3]. Cryoprecipitates were kept ready for cesarean section.
The immediate postpartum patient was kept under strict vigilance in high dependency unit. Strict monitoring of her vital signs was done, and signs and symptoms of abnormal or excessive bleeding were noted. She was transfused the factors daily. Continuous soakage of dressing at the drain site was noted, and ultrasound was done to rule out intra-abdominal collection. Cryoprecipitates were transfused preoperatively. She was taken up for exploratory laparotomy. The bleeding point at drain site was found and ligated. Postpartum period thereafter was uneventful. Furthermore, the baby was strictly monitored and the neonatal period was uneventful. She is regularly following up with hematologists and obstetricians postpartum.
| Discussion|| |
VWD is the most common hereditary coagulopathy with a prevalence of 0.8%–1.3%. An autosomally inherited congenital bleeding disorder, VWD involves a qualitative or quantitative deficiency of VWF, a protein critical for proper platelet adhesion and protection against coagulant factor degradation. Dominant and recessive patterns of inheritance exist. There are three main types of VWD. Type 1 (deficiency of VWF), the most common, is usually mild; type 2 (abnormal VWF) has several subtypes and is less common; and type 3 (absence of VWF), which is rare, is the most severe form.
In VWD, hemostasis is impaired as a result of dual defects in platelet adhesion and in FVIII coagulant activity, the latter requiring VWF as its carrier protein.
The first step in the evaluation of women with suspected bleeding disorders involves obtaining a detailed medical history and performing a physical examination. Women with heavy menstrual bleeding since menarche, postpartum or surgical hemorrhage, plus additional bleeding symptoms, such as bruising, epistaxis, gingival bleeding, or family history of bleeding disorder are considered at risk.
VWF activity has been considered to be the best predictor for risk of bleeding in pregnant women with VWD and bleeding may still occur in women with normal FVIII procoagulant activity. Therefore, it is essential to monitor both VWF antigen and activity levels, together with FVIII procoagulant activity during pregnancy.
The mainstay of treatment for most patients with VWD is desmopressin (1-desamino-8-D-arginine vasopressin). Desmopressin stimulates release of VWF from storage sites, resulting in a transient increase in both VWF and FVIII. However, for patients with qualitative deficiencies (type 2) or severe quantitative deficiencies (type 3), or for patients with milder disease requiring sustained VWF correction, replacement products containing VWF are needed.
Cryoprecipitate, a cold precipitate of plasma enriched in VWF and FVIII, which form the mainstay of treatment of VWD was shown to normalize plasma levels of FVIII, shorten the bleeding time, and stop or prevent bleeding in patients with VWD.
Some plasma-derived FVIII products developed for the treatment of hemophilia A retain relatively intact VWF and have been used successfully in the treatment of VWD. Two such products (Humate-P [CSL Behring, King of Prussia, PA, USA] and Alphanate [Grifols International, S.A., Barcelona, Spain]) were studied prospectively in the treatment of bleeding and prophylaxis for surgery in VWD and have been shown to be highly effective. An individualized approach is suggested for both treatment and prophylaxis in VWD.
| Conclusions|| |
In our patient, there was no diagnostic challenge as the diagnosis of VWD had been established before pregnancy. The challenges that we faced, in this case, were determining when to induce labor, avoiding instrumental delivery so as to minimize blood loss, when to institute cryoprecipitate transfusion and to plan for emergency cesarean section only if there is maternal or fetal indication.
The involvement of MDT members highly specialized in their fields resulted in a successful outcome for both the patient and the neonate.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]