Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Hemostasis > Section 9: Disorders of Secondary Hemo Factor Def > Flashcards

Section 9: Disorders of Secondary Hemo Factor Def Flashcards

1
Q

Hemophilia A is what factor deficiency?

A

FVIII:C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

describe Hemophilia A
how common
inheritance

A
  • most common factor deficiency in USA
  • X-linked recessive expressed almost exclusively in males
  • female carriers generally asymptomatic and produce ~50% of normal VIII
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

hemophilia A mechanism

A
  1. injury to blood vessel -> bleeding
  2. vessel constricts and clotting factors activate
  3. lack of VIII causes weak platelet plug to form
  4. incomplete and/or delayed fibrin clot allows bleeding to continue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

hemophilia A VIII activity ref range

A

50-150%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

hemophilia A mild, moderate, and severe VIII activity

A

mild 5-30% activity
moderate 1-5% activity
severe < 1% activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

hemophilia A lab findings

A
  • bleeding time or PFA normal bc VIII not involved in plt function
  • PT normal
  • APTT prolonged bc VIII intrinsic
  • confirm with factor VIII assay
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

acquired VIII deficiency causes

A
  • DIC bc VIII gets used up
  • liver disease bc not making VIII
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

hemophilia B factor deficiency

A

IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

hemophilia B
history and stats

A
  • royal disease
  • 2nd most common factor deficiency in the USA
  • X-linked recessive almost expressed exclusively in males. Female carriers may have some bleeding problems
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

hemophilia B clinical bleeding

A
  • varying degrees of severity
  • less severe than hemophilia A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

hemophilia B lab findings

A
  • PFA normal
  • PT normal
  • APTT prolonged
  • confirm with factor IX assay
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

acquired factor IX deficiency causes

A
  • DIC
  • liver disease
  • vit K deficiency (IX one of magic 4)
  • oral anticoags (arrest vit K)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

fibrinogen disorder list

A
  • afibrinogenemia
  • hypofibrinogoenemia
  • dysfibrinogenemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

afibrinogenemia

A
  • absence or very low fibrinogen
  • hereditary deficiency
  • bleeding
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

hypofibrinogenemia

A
  • fibrinogen < 100 mg/dl
  • bleeding but depends how low it gets
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

dysfibrinogenemia

A
  • functionally abnormal
  • bleeding if defective clot formation
  • thrombosis if defective fibrinolysis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

acquired fibrinogen deficiency causes

A
  • DIC
  • fibrinogenolysis
  • liver disease
18
Q

hyperfibrinogenemia

A
  • physiologic stresses cause fibrinogen to increase bc it’s an APP (trauma, pregnancy, tissue inflammation)
  • can lead to thrombosis
19
Q

prothrombin deficiency (Factor II)

A
  • rarest of congenital factor deficiencies
  • heterozygotes generally asymptomatic but homozygotes bleed
20
Q

acquired prothrombin deficiency causes

A
  • DIC
  • liver disease
  • vit K deficiency
  • oral anticoags
21
Q

Factor V deficiency

A
  • 1/million population
  • autosomal recessive
  • only homozygotes bleed
  • DIC and liver disease -> acquired
22
Q

Factor VII deficiency

A
  • autosomal recessive
  • only homozygotes bleed
  • DIC/liver disease/vit K def/oral anticoag -> acquired
23
Q

factor X deficiency

A
  • autosomal recessive
  • heterozygotes mild bleeding
  • homozygotes more severe bleeding
  • DIC/ liver disease/vit K def/ oral anticoag/amyloidosis
24
Q

explain amyloidosis in context of factor X deficiency

A

amyloid plaques can adsorb/sequester factor X from being used

25
Q

factor XII deficiency

A
  • relatively common
  • autosomal recessive
  • DIC/liver disease -> acquired
26
Q

factor XII deficiency lab tests

A
  • APTT will be increased but no bleeding problems bc intrinsic pathway affected. Thrombin can go back to activate XI
  • tend to thrombose bc XII involved in plasmin formation
27
Q

prekallekrein deficiency

A
  • rare: mostly in black fams
  • autosomal recessive and dominant
  • APTT increased but no bleeding problems
  • may lead to thrombosis -> anti-thrombotic therapy
28
Q

High Molecular Weight Kininogen (HMWK) deficiency

A
  • autosomal recessive
  • APTT increased, most asymptomatic
  • may lead to thrombosis and need anti-thrombotic therapy
29
Q

factor XIII deficiency

A
  • very rare
  • normal PT and APTT results
  • test with urea solubility (clot lysis)
30
Q

factor XIII deficiency symptoms

A
  • umbilical stump bleeding
  • poor wound healing
  • bruises resolve slowly
  • excessive scar formation
  • cranial hemorrhage
31
Q

what is assumed in disseminated intravascular coagulation (DIC)?

A

resultant secondary fibrinolysis

32
Q

DIC mechanism

A
  • trigger leads to increased clotting, which consumes platelets and clotting factors
  • overactivates fibrinolysis -> too much bleeding and thrombosis
33
Q

primary fibrinolysis lab diagnosis

A
  • PT, APTT, TT, CTT all prolonged
  • FDP positive
  • fibrinogen low
  • D-dimer neg
  • plt count normal
  • peripheral smear normal
34
Q

Why is CTT prolonged in primary fibrinolysis?

A

bc FDPs inhibit thrombin

35
Q

secondary fibrinolysis lab diagnosis

A
  • PT, APTT, TT, CTT all prolonged
  • FDP positive
  • fibrinogen low
  • D-dimer positive
  • plt count low
  • peripheral smear schistocytes
36
Q

which lab tests help distinguish btwn primary and secondary fibrinolysis?

A

D-dimer
plt count
peripheral smear
FDP assay

37
Q

chronic DIC (aka compensated)

A
  • cirrhosis and metastatic cancer
  • less critical bleeding and more diffuse thrombosis
  • proceeds more slowly -> body compensates for loss of factors and plts better
38
Q

liver disease

A
  • liver makes all coag factors, plasminogen, antithrombin, and alpha-2-antiplasmin
  • results in deficiency/dysfunction/impaired clearance of factors/inhibitors
  • accumulate plasminogen activators -> DIC
  • bleeding or thrombosis
39
Q

renal disease

A
  • thrombosis and bleeding
  • nephrotic syndromes (lose some factors, AT, Protein C in urine; uremia pt have decreased plt/impaired plt function)
  • in DIC fibrin deposition results in renal damage
40
Q

vit K deficiency

A
  • factors II, VII, IX, and X vit K dependent
  • Protein C also vit K dependent
41
Q

massive transfusions

A
  • storage of labile factors lose V and VIII which affect screening tests but seldom cause bleeding problems
  • citrate toxicity rarely a problem except in newborns or big transfusions
  • thrombocytopenia -> plt lose viability in stored blood

Hemostasis (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions