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Year 3 - Clinical Science and Professionalism > Haemostasis 2 > Flashcards

Haemostasis 2 Flashcards

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1
Q

what increases the incidence of bleeding disorders

A
  • Consanguinity increases incidence 10-20 times – family history
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2
Q

define consanguinity

A

In clinical genetics, a consanguineous marriage is defined as a union between two individuals who are related as second cousins or closer

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3
Q

Name some rare bleeding disorders

A
  • Factor XI,VII, V, X, II(prothrombin) deficiency
  • Factor XIII and fibrinogen
  • Combined V and VIII, vitamin K dependent factors deficiency
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4
Q

what area is a problem when bleeding

A
  • Mucosal bleeding is more of an issue - therefore menorrhagia is a problem
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5
Q

list the severe platelet disorders

A
  • Glanzmann’s – everything is normal in the assay

- Bernard-Soulier

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6
Q

what does the assay look like in glanzmanns

A

everything is normal in the assay

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7
Q

what does the assay look like in a deficiency of FVII

  • platelet count
  • PT
  • APTT
  • alteration
A
  • platelet count = normal
  • PT = abnormal
  • APTT = normal
  • alteration = coagulation
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8
Q

what does the assay look like in a deficiency of FVIII, FIX, FXI

  • platelet count
  • PT
  • APTT
  • alteration
A
  • platelet count = normal
  • PT = normal
  • APTT = abnormal
  • alteration = coagulation
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9
Q

what does the assay look like in a deficiency of Fibrinogen, FII, FV, FV+VIII, FX

  • platelet count
  • PT
  • APTT
  • alteration
A
  • platelet count = normal
  • PT= abnormal
  • APTT = abnormal
  • alteration = coagulation
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10
Q

what pathways does PT measure

A

extrinsic and common

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11
Q

What pathways does APTT measure

A

Intrinsic and common

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12
Q

for factor XI deficiency what population is predisposed

A
  • Ashkenazi Jewish predisposition

- Genetics – dominant negative effects

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13
Q

what sites of surgery are important in a factor XI deficiency

A
  • Mucosal and urogenital in particular

- Spinal and closed space

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14
Q

What is the treatment of factor XI deficiency

A
  • Factor concentrate – plasma derived
  • Avoid concurrent tranexamic acid – use one or the other (with factor 8 there used together all the time)
  • Aim low normal levels (thrombotic potential)
  • Long half life – alternate day dosing
  • Consider thromboprophylaxis
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15
Q

describe factor VII deficiency

A
  • Poor correlation levels and bleeding tendency – very low levels can be severe
  • Very low levels may be very severe phenotype or coincidental pick up late in life
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16
Q

describe the treatment of factor VII deficiency

A
  • Low dose recombinant fVIIa – 15-30ug/kg
17
Q

what happens to factor VII deficiency in pregnancy

A
  • VII levels may rise in pregnancy – mild/moderate may not need require replacement
  • Possible compensation by other clotting factors rising in pregnancy
  • Assess bleeding phenotype in non-pregnancy state
18
Q

what happens in factor V deficiency

A
  • Generally mild phenotype
  • Don’t need much factor V for haemostasis (>15iu/dl)
  • Some children have early, severe presentation – check VIII
19
Q

How do you treat factor V deficiency

A
  • No concentrate available

- Plasma – solvent detergent FFP – octaplas

20
Q

describe a combined factor V and factor VIII deficiency

A
  • Generally mild phenotype
  • VIII and V genetics normal
  • Abnormality of cellular transport (ERGIC)
21
Q

What are the treatment options for factor V and VIII defficiency

A

VIII concentrate, DDAVP, plasma

22
Q

what are the two types of fibrinogen deficiency

A
  • Quantitative – afibrinogenemia(there is no fibrinogen)/hypofibrinogenemia (reduction in the amount)
  • Qualitative – dysfibrinogenaemia (what you have doesn’t work properly)
  • Coexist – hypodysfibrinogenaemia (both reduction and abnormal function)
23
Q

what is the treatment for fibrinogen deficiency

A

Cryoprecipitate v fibrinogen concentrate

  • Both plasmas derived
  • Cryoprecipitate is not pathogen inactivated
  • Fibrinogen concentrate – heat inactivated
24
Q

What can fibrinogen deficiency care in pregnancy

A
  • Recurrent miscarriage

- Abrupt and PPH

25
Q

How much fibrinogen should you give during pregnancy

A
  • Clearance increases during pregnancy
  • Aim>1.0g/l during pregnancy
  • Aim >2.0g/l for delivery
26
Q

what happens in factor II deficiency

A
  • Variable phenotype

- Think of other vitamin K dependent factors

27
Q

what is the treatment for factor II deficiency

A
  • Treatment = Prothrombin complex concentrate, plasma

- Symptomatic manoeuvres – mirena, COCP etc for menorrhagia

28
Q

what is factor X deficiency and how do you treat it

A
  • Some bleeding correlation with severity level

- May need to consider prophylaxis – prothrombin complex concentrate and plasma

29
Q

what happens in factor XIII deficiency

A
  • Numerical severity often reflects bleeding risk
  • Bleeding symptoms from birth often diagnosed
  • Prolonged umbilical stump bleeding
  • Poor wound healing
30
Q

How do you treat factor XIII deficiency

A
  • Trials of recombinant XIII prophylaxis – market is cardiothoracic surgery (post cardiothoracic surgery bleeding can be a marker of this)
  • Cryo or plasma
31
Q

How do you treat vitamin K deficiency

A
  • Prothrombin concentrate
  • Plasma
  • Check vitamin K given as neonate – haemorrhagic diseases of the newborn
  • Vitamin K is given when they are born to remove the risk of haemorrhagic diseases of the newborn
32
Q

what is glanzmanns disease

A
  • Absent IIb/IIIa receptor on the platelet surface

- Platelets morphologically and numerically normal

33
Q

what does glazmans look like in flow cytometry, platelet function test and platelet aggregometry

A
  • PFA grossly abnormal (needs smaller amount of blood)
  • Platelet aggregometry – absent response to all except ritocetin
  • Flow cytometry – demonstrate absent IIb/IIIa
    Platelet function test is abnormal
34
Q

what happens in Bernard soulier

A
  • Absent Ib/IX/V receptor on platelet surface – VWF receptor

- Platelets morphologically large and reduce in number – have thrombocytopenia

35
Q

what does the

  • PFA
  • platelet aggregometry
  • flow cytometry look like
A
  • PFA abnormal
  • Platelet aggregometry – absent response to ristocetin and others normal
  • Flow cytometry – demonstrate absent Ib/IX/V
36
Q

what is the treatment for both glanzmanns and Bernard souliers

A
  • Platelet – ideal HLA matched/single donor
  • Recombinant VIIa (90mcg/kg)
  • Tranexamic acid
  • COCP
  • Bone marrow transplantation
37
Q

what should you consider when dealing with a patient with blood factor deficiency

A
  • Care around menarche
  • Combined clinic with gynae
  • Contraception
  • Both for bleeding but also unwanted pregnancy
  • Iron deficiency – bleeding and dietary

Year 3 - Clinical Science and Professionalism (32 decks)

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