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MRCOG Maternal Medicine > Bleeding Disorders in Pregnancy GTG > Flashcards

Bleeding Disorders in Pregnancy GTG Flashcards

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1
Q

What is the inherence of haemophilia?

A

X linked recessive condition

Son of female carrier - 50% chance of inheriting
Daughter of female carrier
- 50% chance of being carrier

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2
Q

What clotting factor is absent in haemophilia A and B?

A

A: VIII (8)
B: IX (9)

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3
Q

What % of neonatal males with severe haemophilia have no FHx?

A

50%

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4
Q

What is the plasma concentration of VIII or XI

A

Severe <0.01
Mod 0.01-0.05
Mild 0.06-0.4

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5
Q

Are carriers at risk of bleeding?

A

May still have low factor levels, so should be checked before invasive procedure.

Lionisation (X-chromosome inactivation) can reduce levels in carriers

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6
Q

What pre-pregnancy management/screeening for haemophilia should be offered?

A

Baseline factor level
Optimise health - weight, anaemia

If severe haemophilia carrier:
Offer genetic counselling - ?PGD
Fetal sex determination from 9 weeks
If male foetus, counselling for informed choices
CVS 11-14 weeks

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7
Q

In pregnancy how do levels of factor VIII and factor IX change?

A

Factor VIII increases 2-3 X (reduces risk of bleeding)

Factor IX - no change

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8
Q

When should maternal factor levels be checked?

A

At booking
Before any invasive procedure
3rd trimester

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9
Q

What level should the factor level be for surgical/invasive/spont miscarriage?

A

> 0.5iu/ml

> 1.0 if treatment requires

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10
Q

What treatment should be given if <0.5 and 0.5-1.0

A

<0.5 Recombinant factor/Desmopressin and TXA

0.5-1.0 TXA

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11
Q

What medication is given 1st line to increase factor VIII?

A

Desmopressin (DDVAP) - antidiuretic, fluid restrict 1L in 24hours. Associated Low Na, cannot give in PET

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12
Q

If giving recombinant factor infusion, when to check clotting?

A

Before, after & 4-6hours after

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13
Q

What procedure should be avoided for potentially effected males/female carriers severe?

A

Avoid ECV

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14
Q

What considerations for delivery for haeomphilia?

A

Severe haemophilia male babies - consider LSCS

Avoid: Ventose, midcavity forceps, FBS, FSE

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15
Q

What is the risk of ICH in Haemophilia A and B

A

A 6.2/1000
B 4.1/1000

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16
Q

What concentration must the clotting factors be for insertion/removal of spinal/epidrual/IM injections?

A

> 0.5IU/ml

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17
Q

How long after delivery should clotting factor be maintains >0.5?

A

3 days for VD
5 days for CS

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18
Q

How long should TXA be continued?

A

Until lochia minimal or if CS (7 days)

Lochia has increased duration (21-58 days)

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19
Q

What should thromboprophylaxsis be avoided?

A

If <0.6

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20
Q

What neonatal management should be offered

A

Males born to female carriers - cord blood sample for Dx testing
If low levels - oral vitamin K and pressure following neonate bloodspot test
Consider cranial USS neonates - moderate/severe haemophilia (MRI is symptoms)

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21
Q

For Type 1 VWD
- What type of defect
- Inheritance
- Clinical manifestation

A
  • Partial quantitative (not enough)
  • AD
  • Non severe bleeding

Most common >50%

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22
Q

For Type 2 VWD
- What type of defect
- Inheritance
- Clinical manifestation

A
  • Qualitative (defects)
  • AD
  • Moderate -severe bleeding

Common

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23
Q

For Type 2 A,B,N,M VWD
- What type of defect
- Inheritance
- Clinical manifestation

A
  • Qualitative (defects)
  • AR
  • Moderate -severe bleeding

Uncommon

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24
Q

For Type 3 VWD
- What type of defect
- Inheritance
- Clinical manifestation

A
  • Profound quantitive
  • AR
  • Severe bleeding, clinically similar to haemophilia A

Total or near absence of VW
Rare <5% cases

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25
What happened to VWF levels in pregnancy?
Rise (same as VIII) 2-3 X, usually rectify type 1 VWD
26
Risks of VWF in pregnancy?
APH and PPH 10 fold increase mortality rate No sig increased risk ICH/abpruption
27
When should VWF levels/activity and factor VIII levels be checked?
Booking Before invasive procedures 3rd trimester
28
Where should Type 1/2/3 be managed?
Type 1 (if normal VWF levels) at local unit Type 2/3 - high risk obstetric/haemophilia centre
29
What level should the factor VII and VWF ristocetin cofactor level be for surgical and spont miscarriage?
>0.5
30
If <0.5 what medication can be given?
DDVAP if responsive or VWF containing concentrates If VWF conc given also needs VIII
31
What complication can occur if DDAVP is given to type 2B VWD?
Thrombocytopenia
32
Who should you not give DDVAP?
Type 2B PET Arterial disease Uncontrolled HTN SE: Hypotension, headache, facial flushing
33
Intrapartum management VWD
If <0.5 combination of recombinant factors and TXA 0.5-1.0 just TXA Give Tx near as delivery as possible Check levels pre and post treatment and repeat agentry delivery Type B VWD - platelet transfusion
34
Which procedures should be avoided Type 2/3 VWD?
FBS ECV FSE Ventose Midcavity forceps
35
What analgesia can be offered VWD
Type 1 + normal VWF - can have epidural/spinal Type 2 - avoid unless >0.5 Type 3 avoid Avoid IM and NSAID unless >0.5 (only give short term)
36
How long should VWD/Factor VIII levels be maintained >0.5 PP?
Uncomplicated VD 3 days Instrumental/CS 5 days
37
How long should TXA be continued in VWD?
1g TDS-QDS for 7-14 days
38
Neonatal plan for type 3?
Cord blood if Type 2/3 Type 3 consider routine cranial imaging before discharge, consider factor concentrated if there be potential trauma at delivery Formal testing @ 6 month
39
What population if factor XI deficiency more common?
Ashkenazi Jews 8% heterozygous 0.2-0.5% homozygous
40
Is there a strong correlation with factor levels XI and bleeding?
Poor correlation
41
Does pregnancy effect factor XI levels?
No effect
42
When should factor levels be checked
Booking Before invasive 3rd trimester
43
What does factor XI increase the risk of in pregnancy?
Heavy bleeding after miscarriage PPH Greater if blood group O
44
What are the treatment options for factor XI deficiency?
TXA Factor XI concentrate FFP If non bleeding type - expectant Mgmt is fine
45
Can epidural/spinal be given to those with Factor XI deficiency?
Do not give if: Bleeding phenotype, unknown phenotype, severe reduction in level
46
Neonatal plan for Factor XI deficiency?
No specific plan for neonate
47
What are rare bleeding disorders, how common are they?
Bleeding disorder of a soluble coagulation factor other then VWD & haemophilia A&B. Deficiency fibrinogen, II,V,VII,X,XI and combine V+VIII and congenital deficiency of vitamin K dependant factors. 3-5% inherited coagulation deficiency
48
What is the inheritance of most of the rare inherited bleeding disorders?
Autosomal recessive and heterozygote carriers are usually asymptomatic. Need to ?consanguinity
49
General management of rare bleeding disorders
Similar to above, if severe consider factor replacement +/- TXA. If non severe TXA alone. If severe avoid central neuraxial anaesthesia, LMWH and NSAIDs.
50
What should be given to prothrombin (factor II) deficiency <0.2iu/ml + bleeding in labour/CS?
Give prothrombin complex concentrated 20-40 aiming 0.2-0.4 iu/ml. Continue reduced dose 10-20iu/kg at 48hr intervals for for at least 3 days aiming >0.2.
51
What should be given to factor V deficiency <0.2iu/ml + bleeding in labour/CS?
Give FFP 15-25ml/kg aiming 0.2-0.4 iu/ml, continue at 12 hr intervals for at least 3 days. Severe bleeding consider platelet transfusion
52
What should be given to factor VII deficiency <0.2iu/ml + bleeding in 3rd trismester?
Recombinant factor VIIa 15-30 every 4-6hrs for at least 3-5days following CS. Mild bleeding TXA Severe bleeding as above for 3 doses
53
What should be given to factor x deficiency <0.3iu/ml + bleeding in 3rd trismester or require CS?
Prothrombin complex 20-40 iu/kg aiming >0.4, consider 10-20 every 24 hrs to maintain >0.3 for 3 days
54
What should happened for severe factor XIII deficiency in pregnancy?
Increased intensity of prophylaxis with factor XIII plasma concentrate or recombinant factor XIII. Increase from ever 28 days to every 14-21 days aiming >0.2 For delivery additional factor XIII concentrated 10-40
55
What should be given to factor V+VIII deficiency <0.2iu/ml + in established labour/CS.
SD-FFP 15-25ml/kg aiming factor v activity 0.2-0.4. Consider further SD-FFP 10ml/kg to maintain >0.2 for 3 days. Consider factor VIII if activity <0.5
56
What is the nature of inheritance of afibrinogenaemia, hypofibrinogenaemia and dysfibrinogenamia?
afibrinogenaemia: AR - severe fibrinogen deficiency hypofibrinogenaemia (partial quantitive), dysfibrinogenamia (qualitative) - AD or AR Risk poor wound healing, splenic rupture
57
Fibrinogen disorders are associated with what in pregnancy?
APH, PPH, venous thrombosis, pregnancy loss. afibrinogenaemia - risk ICH and umbilical bleeding
58
If functional fibrinogen <0.5g/litre what should happened in pregnancy?
Fibrinogen concentrate 50-100mg/kg 2 x weekly aiming fibrinogen > 1 In labour aim >1.5 for at least 3 days TXA for minor bleeding If severe - avoid central neuraxial anaesthesia, NSAID, IM injections , avoid midcavity forceps/rotational/ventouse, FBS and FSE.
59
What complication should be carefully considered for fibrinogen replacement?
Risk of thrombosis, wonder LMWH
60
Which are the 2 most severe platelet dysfunction disorders to be aware of?
Bernard Soulier Syndrome (BSS) Glanzmann's thrombasthenia (GT)
61
What abnormality is seen in Bernard Soulier Syndrome, what is its inheritance?
Genetic abnormality of platelet adhesion receptor (GP Ib-IX-V receptor) - severe bleeding AR, heterozygous are asymptomatic, high consanguinity areas.
62
Risk and management of BSS?
Risk Primary/Secondary PPH, wound haematoma Platelet transfusion prophylactically at delivery (HLA matched) + TXA Avoid central neuraxial anaesthesia
63
What is the dysfunction and inheritance of Glanzmanns Thrombasthenia?
Nonfunctinong GP IIb/IIIa mutation, effecting platelet-platelet agrregration. Presents childhood Autosomal recessive
64
Risk and management of Glanzmann's thromboathenia?
Risk PPH If maternal alloimmunisation - risk fetal thrombocytopenia and ICH/fetal bleeding Test for patient specific alloantibodies - booking/28 and 34 weeks HLA matched platelet transfusion and/or recombinant factor VIIa, TXA Avoid central neuraxial anaesthesia If alloimmuisation - refer to fetal medicine, restrictions to delivery, consider ELCS, withhold IM bit K, cord platelet count consider platelet transfusion
65
Inherited bleeding disorder and requesting TOP?
If medium/high risk bleeding - MDT management @ unit with 24 hr blood products, factor replacement and haem advice Correct levels before M/S-TOP. Maintain levels 24hrs post procedure Needs open access to gynae unit/haemophilia centre for weeks after procedure

MRCOG Maternal Medicine (41 decks)

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