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
Reptilase time prolonged in which?
Fibrinogen disorders
25
Lab features of Factor I (fibrinogen) deficiency
Prolonged PT + PTT Prolonged thrombin time + prolonged reptilase time
26
HHT inheritance
Autosomal dominant
27
HHT pathophys
mutations in TGF-B pathway result in excess action of VEGF
28
HHT clinical features
- AVMs - telangiectasias (***See photos online) - epistaxis and GI bleeding - high output cardiac failure from liver AVMs
29
HHT diagnosis
"Curacao criteria" + genetic testing
30
Heyde syndrome triad
- Aortic stenosis - bleeding GI vascular lesion - acquired VWS
31
HHT treatment of bleeding
- IF mild bleeding, antifibrinolytics (TXA, EACA) ablation IF more significant bleeding, bevacizumab oral thalidomide
32
Vascular disorder associated with VWS
- angiodysplasia and GI bleeding (VWF is impt for angiogenesis)
33
Angiodysplasia/GI bleeding classicaly associated with which VWS subtype
Type 2A
34
Treatment of dysfibrinogenemia
Fibrinogen concentrate IF unavailable, cryo
35
Is warfarin safe for breastfeeding patients?
Yes, not present in breast milk
36
Management of Factor XII deficiency
- observation (not a bleeding diathesis)
37
Management of severe VITT
PLEX
38
Benefit of Factor IX Fc fusion
longer half life
39
VWS and aPTT
IF severe, should see some prolongation of PTT and factor VIII deficiency