Bleeding disorders Flashcards

1
Q

Typical clinical presentation of acquired Factor VIII inhibitor

A

Old person w/ Spontaneous mucocutaneous bleeding

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2
Q

Factor XIII deficiency labs

A

Normal coags

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3
Q

Factor XI deficiency 1) clinical features 2) ethnic group

A

1) NO spontaneous bleeding, hemarthrosis, or muslce hematomas
BLeeding typically in setting of trauma or surgery - “injury-related bleeding disorder”
2) Jewish

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4
Q

Other name for factor XI deficiency

A

Hemophilia C

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5
Q

Correlation between Factor XI activity and bleeding manifestations

A

very poor

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6
Q

Treatment of bleeding in Factor XI pts

A

Minor bleeding:
Antifibrinolytics (mainstay)
Severe bleeding:
rFVIIa
*Factor XI concentrate not available in US

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7
Q

Factor VII deficiency inheritance pattern

A
  • autosomal recessive
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8
Q

Factor VII clinical feature to remember

A

thrombotic tendency

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9
Q

Factor X deficiency inheritance

A

autosomal recessive

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10
Q

Correlation of Factor level with bleeding phenotype in Factor X

A

very good

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11
Q

Clinical conditions associated with factor X

A

Plasma cell dyscrasias

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12
Q

Factor X deficiency treatment

A

FX concentrate
IF not available, PCC vs. FFP
Treatment of plasma cell dyscrasia

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13
Q

Condition associated with Factor V deficiency

A

Quebec platelet disorder

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14
Q

Factor V deficiency treatment

A

1) FFP
2) Platelet transfusions (platelets have Factor V in alpha granules)

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15
Q

Factor V association

A

Topical Bovine thrombin

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16
Q

Factor XIII deficiency coags

A

normal

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17
Q

Factor XIII deficiency management

A

Recombinant FXIII A subunit product
IF not available, FFP or cryo
IF <10%, FXIII prophylaxis monthly due to ICH risk

17
Q

Factor XIII deficiency clinical features

A

Delayed bleeding + ***umbilical stump bleeding as neonates + **Intracerebral hemorrhage + pregnancy loss

18
Q

Pts at risk of developing prothrombin deficiency

A

APLS pts (

19
Q

Treatment of acquired prothrombin deficiency (factor II deficiency)

A

Prothrombin complex concentrates (PCCs)

20
Q

What is FEIBA?

A

activated prothrombin complex concentrate

21
Q

What is Factor I

A

fibrinogen

22
Q

Classical clinical vignette for fibrogen deficiency

A

Umbilical stump bleeding as a neonate

23
Q

Clinical feature of dysfibrinogenema (qualitiative) to remember

A

Prothrombotic

24
Q

Reptilase time prolonged in which?

A

Fibrinogen disorders

25
Q

Lab features of Factor I (fibrinogen) deficiency

A

Prolonged PT + PTT
Prolonged thrombin time + prolonged reptilase time

26
Q

HHT inheritance

A

Autosomal dominant

27
Q

HHT pathophys

A

mutations in TGF-B pathway result in excess action of VEGF

28
Q

HHT clinical features

A
  • AVMs
  • telangiectasias (***See photos online)
  • epistaxis and GI bleeding
  • high output cardiac failure from liver AVMs
29
Q

HHT diagnosis

A

“Curacao criteria” + genetic testing

30
Q

Heyde syndrome triad

A
  • Aortic stenosis
  • bleeding GI vascular lesion
  • acquired VWS
31
Q

HHT treatment of bleeding

A
  • IF mild bleeding,
    antifibrinolytics (TXA, EACA)
    ablation
    IF more significant bleeding,
    bevacizumab
    oral thalidomide
32
Q

Vascular disorder associated with VWS

A
  • angiodysplasia and GI bleeding (VWF is impt for angiogenesis)
33
Q

Angiodysplasia/GI bleeding classicaly associated with which VWS subtype

A

Type 2A

34
Q

Treatment of dysfibrinogenemia

A

Fibrinogen concentrate
IF unavailable, cryo

35
Q

Is warfarin safe for breastfeeding patients?

A

Yes, not present in breast milk

36
Q

Management of Factor XII deficiency

A
  • observation (not a bleeding diathesis)
37
Q

Management of severe VITT

A

PLEX

38
Q

Benefit of Factor IX Fc fusion

A

longer half life

39
Q

VWS and aPTT

A

IF severe, should see some prolongation of PTT and factor VIII deficiency