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CSP1 > Haemostasis 2 > Flashcards

Haemostasis 2 Flashcards

1
Q

Examples of rare bleeding disorders

A
  • factor XI, VII, V, X, II (prothrombin) deficiency
  • factor XIII and fibrinogen
  • combined V & VIII, vit K dependent factors deficiency
  • Glanzmanns
  • Bernard Soulier
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2
Q

Features of rare bleeding disorders

A
  • CNS, GI, haemarthroses, haematomas less frequent
  • mucosal bleeding
  • menorrhagia in 50%
  • broadly less life/limb threatening
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3
Q

FVII deficiency Blood Count

A

Abnormal PT

Normal APTT

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4
Q

FVII, FIX, FXI deficiency blood count

A

Normal PT

Abnormal APTT

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5
Q

Fibrinogen, FII, FV, FV + VIII, FX deficiency blood count

A

Abnormal PT

Abnormal APTT

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6
Q

Features of factor XI deficiency

A
  • complicated genetics
  • severity doesn’t correlate with level of factor
  • mucosal and urogenital, spinal and closed space sites of surgery
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7
Q

Treatment of factor XI deficiency

A
  • plasma derived factor concentrate
  • avoid concurrent tranexamic acid
  • aim low normal levels
  • long half life
  • consider thromboprophylaxis
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8
Q

Features of factor VII deficiency

A
  • poor correlation levels and bleeding tendency
  • if very low levels = severe phenotype or coincidental pick up late in life
  • low dose recombinant fVIIa
  • VII may rise in pregnancy
  • possible compensation by other clotting factors rising in pregnancy
  • assess bleeding phenotype in non-pregnant state
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9
Q

Features of factor V deficiency

A
  • generally mild
  • early, severe presentation
  • check VIII level
  • no concentrate available
  • plasma (solvent detergent FFP)
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10
Q

Factor V and VIII deficiency

A
  • generally mild
  • VIII and V genetics
  • treatment = VIII concentrate, DDAVP, plasma
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11
Q

Fibrinogen deficiency

A
  • bleeding and thrombotic risks
  • quantitative = afibrinogenaemia/hypofibrinogenaemia
  • qualitative = dysfibrinogenaemia
  • coexist = hypodysfibrinogenaemia
  • potential severe bleeding
  • umbilical cord bleeding
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12
Q

Difference between cryoprecipitate vs fibrinogen concentrate

A
  • both for fibrinogen deficiency
  • both plasma derived
  • cryo not pathogen inactivated
  • fibrinogen concentrate activated by heat
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13
Q

Causes of fibrinogen deficiency in pregnancy

A

Recurrent miscarriage

Abruption and PPH

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14
Q

Fibrinogen replacement in pregnancy

A
  • clearance increases during pregnancy
  • aim >1g/l during pregnancy
  • aim >2g/l for delivery
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15
Q

Factor II deficiency

A
  • variable phenotype
  • prothrombin complex conc.. and plasma
  • COCP for menorrhagia
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16
Q

Factor X deficiency

A
  • some bleeding correlation with severity level

- may need to consider prophylaxis = prothrombin complex conc. and plasma

17
Q

Factor XIII deficiency

A
  • poor wound healing
  • prolonged umbilical stump bleeding
  • bleeding symptoms from birth
  • numerical severity often reflects bleeding risk
  • conventional bleeding test will not detect
  • trials of recomb XIII for prophylaxis
  • cryo or plasms
18
Q

Vit K Dependent factor deficiency

A
  • factors II, VII, IX and X
  • PT concentrate
  • plasma
  • check vit K given as neonate otherwise haemorrhagic disease of newborn
19
Q

Which rare bleeding disorders don’t have a mutation in corresponding clotting factor gene

A
  • combined V and VIII deficiency

- vit K dependent factor deficiency

20
Q

Features of Glanzmanns

A
  • absent IIb/IIIa receptor on platelet surface
  • platelets morphologically and numerically normal
  • PFA grossly abnormal
  • platelet aggregometry = absent response to all except ristocetin
  • flow cytometry = demonstrate absent IIb/IIIa
21
Q

Features of Bernard-Soulier

A
  • absent Ib/IX/V receptor on platelet surface
  • platelets morphologically large and reduced in number
  • PFA normal
  • platelet aggregometry shows absent response to ristocetin
  • flow cytometry demonstrates absent Ib/IX/V
22
Q

Treatment options for Glanzmanns and Bernard SOulier

A
  • platelet ideal HLA matched
  • recomb VIIa
  • tranexamic acid
  • COCP
  • bone marrow transplantation
  • HLA and anti receptor ab
  • care around menarche
  • combined clinic ith gynae
  • contraception
  • iron deficiency
  • bleeding
  • dietary

CSP1 (20 decks)

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