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MLS Classes > Coag U4 - Hemorrhagic Disorders > Flashcards

Coag U4 - Hemorrhagic Disorders Flashcards

1
Q

hemorrhage

A

excessive bleeding that needs medical or physical intervention

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2
Q

localized bleeding

A

bleeding from one location, like from an injury, infection, or tumor

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3
Q

what are some examples of localized bleeding?

A

ineffective sutured surgical site or arteriovenous malformations (AMV)

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4
Q

generalized bleeding

A

bleeding from multiple sites, spontaneous/recurring bleeds, or hemorrhaging that needs physical intervention

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5
Q

what is an example of generalized bleeding?

A

disorders relating to primary or secondary hemostasis

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6
Q

mucocutaneous hemorrhaging

A

bleeding in the skin or at a body orifice

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7
Q

what are some examples of mucocutaneous bleeds?

A

petechiae, purpura, ecchymoses, defects of primary hemostasis

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8
Q

anatomic hemorrhaging

A

bleeding in the soft tissue, muscles, joints, or deep in the tissues

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9
Q

what are some examples of anatomical bleeds?

A

after minor trauma, dental extractions, surgery
internal bleeds within the joints, body cavities, muscles
defects of secondary hemostasis

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10
Q

what is the difference between congenital and acquired bleeding disorders?

A

congenital is genetic, the first sign is bleeding from the umbilical cord
acquired develops after a disease or physical trauma

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11
Q

in order, what are the most common congenital deficiencies?

A

VWF, factor 8 (hemophilia A), factor 9 (hemophilia B)

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12
Q

trauma-induced coagulopathy (TIC)

A

accounts for most instances of fatal hemorrhage

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13
Q

what is the first coag factor that decreases activity in liver disease?

A

factor 7

prolonged PT is an early marker for liver disease because it is sensitive

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14
Q

what does decrease in factor 10 mean?

A

another marker for liver disease

more specific indicator because it is not vitamin K dependent

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15
Q

what would the presence of fibrinogen mean in liver disease?

A

increases in early mild liver disease and is an acute phase reactant

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16
Q

dysfibrinogenemia

A

fibrinogen coated with excessive sialic acid, fibrinogen functions poorly

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17
Q

what would you expect to happen to VWF, factor 8, factor 13 in liver disease?

A

will be unaffected or elevated in mild/moderate liver disease, are acute phase reactants

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18
Q

what do the presence of factor 10 and factor 12 do in liver disease?

A

helps in differentiating liver disease from vitamin K deficiency

both factors are decreased in liver disease
factor 10 - normal in vitamin K deficiency
factor 12 - decrease in vitamin K deficiency

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19
Q

what test confirms dysfibrinogenemia?

A

reptilase time test

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20
Q

what is chronic renal failure associated with?

A

associated with PLT dysfunction and mucocutaneous bleeding

PLT adhesion and aggregation are suppressed

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21
Q

dialysis

A

temporarily activates PLTs and improves PLT function

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22
Q

desmopressin acetate

A

increase VWF multimers which aids PLT adhesion and aggregation

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23
Q

coumadin/warfarin

A

disrupts enzymes involved in vitamin K activation of coag factors

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24
Q

what does proteins induced by vitamin K antagonists (PIVKA) do?

A

inactivate factors 2, 7, 9, 10, and proteins C, S, Z

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25
Q

how does vitamin K deficiency lead to bleeding issues?

A

activates coag factors → fibrin → fibrin clot

26
Q

what is autoanti-factor 8 most commonly seen in? what is it also known as?

A

acquired hemophilia, a factor inhibitor

27
Q

what are some notable associations with acquired hemophilia?

A

patients older than 60
associated with RA, IBD, SLE
patients prescribed immunosuppressive therapy
those who experience sudden and severe bleeding in soft tissues, GI, genitourinary tract

28
Q

type 1 VWD

A

quantitative deficiency
makes up 40-70% of VWF cases - therefore most common
mild to moderate systemic bleeding and menorrhagia

29
Q

type 2 VWD

A

qualitative VWF abnormalities (five types 2A, 2B, 2M, 2N, 3)
VWF function consistently reduced

30
Q

in VWF, how do CBC and PT and PTT play a role?

A

CBC - rules out thrombocytopenia
PT and PTT - assess coag cascade to rule out other coag factor deficiencies

31
Q

VWF: RCo

A

add to patient plasma → unfolds the VWF molecule and reduces repelled negative charges → HMW-VWF multimers bind reagent PLT membrane GPIb/IX/V receptors

32
Q

VWF: Ag assay

A

most important in VWF lab profile
EIA methodology, LIA/Stago methodology, CLIA technology

33
Q

factor 8 assay

A

markedly reduced in VWF (for subtype 2N)

34
Q

what will lead to VWF collagen-binding (VWF:CB) assay?

A

poor reproducibility of VWF: RCo

35
Q

hemophilias

A

deficiencies associated with anatomic soft tissue bleeding
the second most common congenital bleeding disorder
mostly occurring in males

36
Q

what is hemophilia A a deficiency for?

A

factor 8, aka Classic hemophilia

37
Q

why does factor 8 deficiency lead to hemorrhagic results?

A

it significantly slows the coag pathway’s production of thrombin

38
Q

structure of factor 8

A

two-chained, 285,000-Dalton protein translated on the X chromosome

39
Q

normal function of factor 8

A

thrombin cleaves factor VIII →
B domain large polypeptide is released →
calcium-dependent heterodimer is left behind that detaches from VWF to create the tenase complex →
massive activation of factor X

40
Q

genetics of hemophilia A

A

located on the X chromosome
affected males will experience anatomic bleeding
female carriers/heterozygotes do not experience bleeding
approximately 30% of newly diagnosed patients have no family history of hemophilia A

41
Q

what are the genotypes of an carrier mother and an unaffected father?

A

25% unaffected daughter
25% carrier/heterozygote daughter
25% unaffected son
25% affected/hemophilic son

42
Q

what are the genotypes of an unaffected mother and an affected (with hemophilia A) father?

A

50% carrier/heterozygote daughters
50% unaffected sons

43
Q

anatomical bleeds of hemophilia A

A

deep muscle and joint hemorrhages, hematomas, wounds oozing after trauma or surgery, bleeding into CNS, peritoneum, GI tract, and kidneys

44
Q

diagnosing newborns

A

perform lab testing on babies with mothers that have a history of hemophilia
abnormal bleeding (easy bruising, bleeding from umbilical stump, hematuria, intracranial bleed) is suspicious for hemophilia

45
Q

complications with hemophilia A

A

debilitate and progressive musculoskeletal lesions and deformities
neurologic deficiencies leading to intracranial
hemorrhage

46
Q

in hemophilia A, what do the screening tests look like?

A

PT, FBG, TT - normal
PTT - prolonged

47
Q

hemophilia A therapy/treatment

A

use self-administered desmopressin acetate (DDAVP)

“on-demand” treatment - raise FVIII activity
when a patient suspects a bleed or anticipates a hemostatic challenge

48
Q

Nijmegen-Bethesda assay (titer)

A

quantitative inhibitor

49
Q

which population is most affected by hemophilia A and why?

A

males because they only have one X chromosome, making them more likely to be affected

50
Q

what is hemophilia B a deficiency for?

A

factor 9, aka Christmas Disease

51
Q

pathophysiology of hemophilia B

A

reduces thrombin production, causing soft tissue anatomic bleeding

52
Q

lab tests of hemophilia B

A

PT, FBG, TT - normal
PTT - prolonged

53
Q

treatment of hemophilia B

A

purified factor 9 concentrates, repeat doses every 24 hours

54
Q

in what percentage of patients have inhibitors?

A

3%

55
Q

what is hemophilia C a deficiency for?

A

factor 11, aka Rosenthal syndrome

56
Q

facts about hemophilia C

A

autosomal dominant
mild to moderate bleeding symptoms
more than half cases seen in Ashkenazi Jews

57
Q

lab tests of hemophilia C

A

PT - normal
PTT - prolonged

58
Q

treatment of hemophilia C

A

frequent plasma infusions

59
Q

VWF primary function

A

mediate PLT adhesion to subendothelial collagen in areas of high flow rate and shear force

60
Q

review question: which factors are associated with the intrinsic pathway?

A

XII, XI, IX, VIII

61
Q

review question: which factors are associated with the extrinsic pathway?

A

VII

62
Q

review question: which factors are associated with the common pathway?

A

I, II, V, X

MLS Classes (13 decks)

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