27
yrs Mrs. XY, primi para with 16 weeks of pregnancy, was referred to
us by haematologist. She was diagnosed as suffering from VWD Type 3
at the age of 5 years, when she presented with delayed clotting of blood
and bruising at various sites on and off.
Her family history revealed that both of her sisters had same disease
whereas both the parents and brothers were not affected.
Patient was kept under strict antenatal surveillance. Her clotting profile
at the time of her first antenatal visit i.e. at 16 weeks showed; BT
> 20 min, and APTT = 60 (control 130), Factor VIII : C = 3%; VWF
= 5%, VWF : RCOF = 1%, VWF : AG = 1%. Haemoglobin and other routine
blood investigations were within normal limits.
Serial sonography scan showing healthy growth, frequent antenatal check
ups, CBC and clotting profile were done periodically throughout the
course of pregnancy. NSAID and IM injections were completely avoided.
Patient was advised to seek medical help immediately if any bleeding
episode took place.
In view of limited facilities for procuring cryoprecipitate in emergency
situation, and to avoid any untoward bleeding catastrophe, vaginal delivery
was not tried and decision of elective caesarean section with bilateral
tubal ligation was taken as soon as it becomes near team pregnancy with
adequate foetal maturity.
She was admitted at 37 weeks with coagulation profile showing:
BT > 20 min, APTT = 83 (control = 30), VWF = 2%, VWF : AG = 0% (50-150%),
VWF: RCOF = 0% (50-150%), Hb = 12.2%, Platelet Count = adequate.
On Examination general condition was fair, vitals and blood pressure
were within normal limits. Per abdomen; uterus was term, with vertex
presentation and floating head. Per vaginal examination; os closed,
uneffaced. NST showed reactive pattern.
According to haematologist's advise 15 bags of cryoprecipitate (each
cryoprecipitate concentrate consist of factor VIII 80-120 units, fibrinogen
50-250 mg, VW factor 40-70% of original FFP, factor XIII 20-30% of original
FFP) were transfused 6 hours before surgery. Inj. Trenaxa 5 ml in 100
ml of normal saline (NS) was given 2 hours before surgery.
Elective caesarean section with bilateral tubal ligation under general
anaesthesia was performed. A 3.2 kg live male child was delivered with
APGAR of 8/10. Baby was kept in Neonatal ICU for observation. VW factor
and coagulation profile of baby were normal. In post-operative period,
the patient was transfused with 15 bags of cryoprecipitate twice daily
for 3 days, followed by 10 bags BD for 2 days, followed by 5 bags BD
for 2 days. Inj. Minirin and Inj trenaxa (5 ml in 100 ml of NS) were
given twice daily for 6 days. Post-operative period was uneventful.
Baby and mother were discharged on 10th post operative day. Patient
followed up after 2 weeks.
Thus with a multidisciplinary approach involving obstetrician, haematologist
and neonatologist a healthy maternal and foetal outcome was achieved.
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on
Willebrand disease is a disorder resulting from quantitative or qualitative
deficiency in Von-Willebrand factor which is necessary for platelet
adhesion and factor VIII stabilization.
VWD is most frequent bleeding disorder with estimated prevalence of
1%, but the prevalence of cases with significant bleeding symptoms requiring
treatment in about 2-3/million. Type 1 (milder), is autosomal dominant
comprising 70-80% of cases, characterized by decreased VW factor, Type
2 also autosomal dominant, where assembly of VW factor is defective
(qualitative). Type 3 is autosomal recessive form which is rarest and
most severe form of disease, where VW factor is severely decreased or
absent.1 VWD is characterized by increased bleeding time and activated
partial thromboplastin time with normal platelet count.
VWD is confirmed by factor VIII : C, VWF : AG, VWF : RCOF activity in
blood. To determine type : Ristocetin induced platelet agglutination;
plasma VWF multimer analysis is useful.2
Incidence of post partum haemorrhage is around 30% and it may be extremely
severe. PPH risk is especially increased 24 hours after delivery.3 There
has been report of haemoperitoneum in pregnancy in patient with VBD
Type 3.4
Main line of treatment consists of post-partum replacement of required
blood component along with effective uterotonic therapy to be continued
for 7 days post-partum. Plasma concentrates containing factor VIII and
VWF treated with virucidal methods are safe and treatment of choice.5
But in 3rd world countries due to restricted resources cryoprecipitate
and fresh frozen plasma are treatment of choice.
There is virtually no place for DDAVP in obstetric; perhaps it may be
used in puerperium.6
There are some trials suggesting continuous infusion of VBD factor i.e.
haemate-P solution in patients of VBD as safe and effective treatment
in post-operative period.7
|
| 1. |
Rodghiro
F. Von Willebrand disease : still intriguing disorder in the
era of molecular medicine. Haemophilia 2002; 8 (3) : 292. |
| 2. |
Guthu
U, T Sakris DA, Hosli I. Management of patient with type 2B
von willebrands disease during delivery and peurperium. Z Gebur
Shilfe Neonatol 2002; 204 (4) : 151-5. |
| 3. |
Federic
AB, Mannucci PM. Advances in the genetic and treatment of von
willebrand disease. Curr Opinion Pediatric 2002; 14 (1) : 22-33. |
| 4. |
Pommie
C, Perrin C, Dorne R, De Roissuel JP, Arnold P. Haemoperitonium
and pregnancy in patient with von willebrand disease type 3.
Ann Fr Anesth Reanim 2002; 21 (5) : 436-9. |
| 5. |
Jaquies
Wallach. Interpretation of diagnostic tests 7th edition. p:
487-87. |
| 6. |
Michael
de Swiet. Medical Disorder in obstetric practice. 3rd edition.
p: 101-104. |
| 7. |
Lubetsky
A, Schulman S, Varon D, et al. Safety and efficacy of continuous
infusion of combined factor VIII - Von Willebrand factor (VWF)
concentrate (Haemate - P) in patients with Von Willebrand disease.
Thromb Haemost 1999; 81 (2) : 229-33. |
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