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MRCOG Maternal Medicine > Bleeding Disorders in Pregnancy GTG > Flashcards

Bleeding Disorders in Pregnancy GTG Flashcards

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
Q

What happened to VWF levels in pregnancy?

A

Rise (same as VIII) 2-3 X, usually rectify type 1 VWD

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26
Q

Risks of VWF in pregnancy?

A

APH and PPH
10 fold increase mortality rate
No sig increased risk ICH/abpruption

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27
Q

When should VWF levels/activity and factor VIII levels be checked?

A

Booking
Before invasive procedures
3rd trimester

28
Q

Where should Type 1/2/3 be managed?

A

Type 1 (if normal VWF levels) at local unit
Type 2/3 - high risk obstetric/haemophilia centre

29
Q

What level should the factor VII and VWF ristocetin cofactor level be for surgical and spont miscarriage?

A

> 0.5

30
Q

If <0.5 what medication can be given?

A

DDVAP if responsive or VWF containing concentrates

If VWF conc given also needs VIII

31
Q

What complication can occur if DDAVP is given to type 2B VWD?

A

Thrombocytopenia

32
Q

Who should you not give DDVAP?

A

Type 2B
PET
Arterial disease
Uncontrolled HTN

SE: Hypotension, headache, facial flushing

33
Q

Intrapartum management VWD

A

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
Q

Which procedures should be avoided Type 2/3 VWD?

A

FBS
ECV
FSE
Ventose
Midcavity forceps

35
Q

What analgesia can be offered VWD

A

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
Q

How long should VWD/Factor VIII levels be maintained >0.5 PP?

A

Uncomplicated VD 3 days
Instrumental/CS 5 days

37
Q

How long should TXA be continued in VWD?

A

1g TDS-QDS for 7-14 days

38
Q

Neonatal plan for type 3?

A

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
Q

What population if factor XI deficiency more common?

A

Ashkenazi Jews
8% heterozygous
0.2-0.5% homozygous

40
Q

Is there a strong correlation with factor levels XI and bleeding?

A

Poor correlation

41
Q

Does pregnancy effect factor XI levels?

A

No effect

42
Q

When should factor levels be checked

A

Booking
Before invasive
3rd trimester

43
Q

What does factor XI increase the risk of in pregnancy?

A

Heavy bleeding after miscarriage
PPH

Greater if blood group O

44
Q

What are the treatment options for factor XI deficiency?

A

TXA
Factor XI concentrate
FFP

If non bleeding type - expectant Mgmt is fine

45
Q

Can epidural/spinal be given to those with Factor XI deficiency?

A

Do not give if: Bleeding phenotype, unknown phenotype, severe reduction in level

46
Q

Neonatal plan for Factor XI deficiency?

A

No specific plan for neonate

47
Q

What are rare bleeding disorders, how common are they?

A

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
Q

What is the inheritance of most of the rare inherited bleeding disorders?

A

Autosomal recessive and heterozygote carriers are usually asymptomatic. Need to ?consanguinity

49
Q

General management of rare bleeding disorders

A

Similar to above, if severe consider factor replacement +/- TXA. If non severe TXA alone.
If severe avoid central neuraxial anaesthesia, LMWH and NSAIDs.

50
Q

What should be given to prothrombin (factor II) deficiency <0.2iu/ml + bleeding in labour/CS?

A

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
Q

What should be given to factor V deficiency <0.2iu/ml + bleeding in labour/CS?

A

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
Q

What should be given to factor VII deficiency <0.2iu/ml + bleeding in 3rd trismester?

A

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
Q

What should be given to factor x deficiency <0.3iu/ml + bleeding in 3rd trismester or require CS?

A

Prothrombin complex 20-40 iu/kg aiming >0.4, consider 10-20 every 24 hrs to maintain >0.3 for 3 days

54
Q

What should happened for severe factor XIII deficiency in pregnancy?

A

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
Q

What should be given to factor V+VIII deficiency <0.2iu/ml + in established labour/CS.

A

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
Q

What is the nature of inheritance of afibrinogenaemia, hypofibrinogenaemia and dysfibrinogenamia?

A

afibrinogenaemia: AR - severe fibrinogen deficiency

hypofibrinogenaemia (partial quantitive), dysfibrinogenamia (qualitative) - AD or AR

Risk poor wound healing, splenic rupture

57
Q

Fibrinogen disorders are associated with what in pregnancy?

A

APH, PPH, venous thrombosis, pregnancy loss.

afibrinogenaemia - risk ICH and umbilical bleeding

58
Q

If functional fibrinogen <0.5g/litre what should happened in pregnancy?

A

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
Q

What complication should be carefully considered for fibrinogen replacement?

A

Risk of thrombosis, wonder LMWH

60
Q

Which are the 2 most severe platelet dysfunction disorders to be aware of?

A

Bernard Soulier Syndrome (BSS)
Glanzmann’s thrombasthenia (GT)

61
Q

What abnormality is seen in Bernard Soulier Syndrome, what is its inheritance?

A

Genetic abnormality of platelet adhesion receptor (GP Ib-IX-V receptor) - severe bleeding
AR, heterozygous are asymptomatic, high consanguinity areas.

62
Q

Risk and management of BSS?

A

Risk Primary/Secondary PPH, wound haematoma

Platelet transfusion prophylactically at delivery (HLA matched) + TXA

Avoid central neuraxial anaesthesia

63
Q

What is the dysfunction and inheritance of Glanzmanns Thrombasthenia?

A

Nonfunctinong GP IIb/IIIa mutation, effecting platelet-platelet agrregration. Presents childhood

Autosomal recessive

64
Q

Risk and management of Glanzmann’s thromboathenia?

A

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
Q

Inherited bleeding disorder and requesting TOP?

A

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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