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HEME/LYMPH Exam 2 > RBC and Bleeding Disorders VII > Flashcards

RBC and Bleeding Disorders VII Flashcards

1
Q

inherited disorders of platelet function

A

defects of adhesion
defects of aggregation
disorders of platelet secretion

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2
Q

bernard soulier

A

defect in Ib-IX
-vWF receptor

severe bleeding tendency

defectiv in platelet adhesion

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3
Q

glanzmann thrombasthenia

A

auto recessive
defective IIb-IIIa

no bridging of platelet

defect in aggregation

severe bleeding tendency

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4
Q

disorders of platelet secretion

A

defective release of thromboxane and ADP

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5
Q

acquired defect in platelet function

A

aspirin and NSAIDs

cox inhibitors

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6
Q

uremia

A

acquired defect in platelet function

adhesion granule secretion and aggregation

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7
Q

arachidonic acid metabolism

A

localized endothelial metabolic sequence associated with tendency to limit bleeding/vasodilation

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8
Q

intrinsic pathway

A

starts with factor XII

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9
Q

extrinsic pathway

A

starts with tissue factor to factor VII

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10
Q

prothrombin to thrombin

A

Xa

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11
Q

thrombin activity

A

cleaves fibrinogen to fibrin

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12
Q

clotting in vivo

A

initiated mainly by tissue factor

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13
Q

Hem A

A

factor VIII

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14
Q

Hem B

A

factor IX

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15
Q

large posttraumatic ecchymoses or hematomas, prolonged bleeding after surgery/laceration

A

coag factor deficiency

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16
Q

GI bleeds, GU bleeds, hemarthrosis

A

coag factor deficiency

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17
Q

patient oozes blood days after tooth extraction

A

coag factor deficiency

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18
Q

most common deficiency in coag factors

A

Hem A, Hem B, vWD

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19
Q

vit K deficiency

A

impaired synthesis of 2, 7, 9, 10 and protein C

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20
Q

DIC

A

consumption of multiple coag factors

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21
Q

two most common inherited bleeding disorders

A

Hem A and vWD

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22
Q

synthesis of factor VIII

A

sinusoidal endo cells and kupffer cells in liver

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23
Q

factor VIII in circulation

A

binds vWF

stabilizes and extends half life from 2 hours to 12 hours

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24
Q

ristocentin agglutination test

A

measure of vWF function

causes clumping

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25
Q

inital binding of vWE to endothelial

A

GpIb

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26
Q

bridging of fibrinogen in platelet aggregation

A

GpIIb/IIIa

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27
Q

most common inherited bleeding disorder of humans

A

vWD

mild bleeding tendency - surgery or dental procedure

spontaneuous epistaxis

auto domimant

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28
Q

three categories of vWD

A

type 1 and 3 - quantitative

type 2 - qualitative

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29
Q

type I vWD

A

auto dominant
-quantitative defect

70% of cases

mild disease

point substitutions
-rapid clearance from plasma

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30
Q

type III vWD

A

auto recessive
-quantitative defect

severe clinically - very low vWF

effects factor VIII stability

deletions or frameshift mutations of both alleles

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31
Q

type II vWD

A

qualitative defect

-2A - most common form

auto dominant

missense mutations - defective multimer assembly

25% of cases

mild to moderate bleeding

32
Q

vWD clinical

A

prolonged PTT - due to lack of factor VIII stability

reduced vWF - measured by ristocetin

33
Q

desmopressin

A

tx of vWD patient undergoing surgery or dental work

causes vWF release

34
Q

most common hereditary disease associated with life-threatening bleeding

A

Hemophilia A

mutation in factor VIII

X-linked recessive

affects mainly males

35
Q

hem A

A

mutation in factor VIII

X-linked recessive

affects mainly males

rarely in females - inactivation- unfavorable lyonization

36
Q

<1% Factor VIII

A

severe Hem A

37
Q

2-5% factor VIII

A

moderately severe Hem A

38
Q

> 6% factor VIII

A

mild disease Hem A

39
Q

most severe deficiency of factor VIII

A

involve inversion involving X-chromosome that completely abolishes the synthesis of factor VIII

40
Q

easy bruising, massive hemorrhage after trauma, hemarthroses, NO petechiae

A

hem A

41
Q

prolonged PTT and normal PT

A

hem A

defect in intrinsic pathway

42
Q

diagnosis of Hem A

A

factor VIII specific assays

43
Q

Tx Hem A

A

infusion of recombinant factor VIII

15% patients with severe disease- develop Abs that bind factor VIII

also risk of HIV transfusion has been almost eliminated

44
Q

christmas disease

A

Hem B

factor IX deficiency

clinically indistinguishable from Hem A

X-linked recessive

PTT prolonged and PT normal

45
Q

diagnosis of christmas disease

A

only possibly by assay of factor levels

46
Q

tx of Christmas disease

A

infusion of recombinant factor IX

47
Q

hypoxia and infarction as well as hemorrhage

A

DIC

deposition of microthrombi as well as depletion of heme factors

48
Q

DIC

A

not primary disease

coagulopathy occuring in course of variety of clinical condition

49
Q

activation of factor X

A

generation of thrombin

thrombin converts fibrinogen to fibrin

50
Q

thrombomodulin

A

binds and inactivates thrombin

this complex binds and activates protein C
-inhibitor of factors V and VIII

51
Q

mechanisms triggering DIC

A

widespread injury to endo cells

release of tissue factor

52
Q

mucus from adenocarcinomas

A

procoagulant - can initiate DIC

53
Q

TNF

A

induces endo cells to express tissue factor - can lead to sepsis

54
Q

causes of CID

A

most likely obstetric complications
malignant neoplasms
sepsis
major trauma

55
Q

bacterial infection

A

endotoxins inhibit expression of thrombomondulin

-to DIC

56
Q

massive trauma

A

release of tissue factor

-to DIC

57
Q

consequences of DIC

A

deposition of fibrin within microcirculation
-can lead to ischemia and microangiopathic hemolytic anemia

consumption of platelets and clotting factors - hemorrhagic diathesis

58
Q

plasmin

A

cleaves fibrin and digests factor V and VIII

59
Q

bilateral renal cortical necrosis

A

imcroinfarcts from DIC

60
Q

waterhouse friedrishsen syndrome

A

massive adrenal hemorrhage

-with DIC from meningococcemia infection

61
Q

kasabasch merritt syndrome

A

form of DIC with giant hemangiomas

thrombin form with neoplasm

62
Q

DIC clinical

A

acute - endotoxic shock or amniotic fluid embolism
chronic - carcinomatosis or retention of dead fetus

50% are obstetric patient with pregnancy complications

disorder reversible with delivery of fetus

33% carcinomatosis

63
Q

microangiopathic hemolytic anemia, dyspnea, cyanosis, resp failure, convulsions coma, oliguria, acute renal failure, circ failure and shock

A

DIC

64
Q

acute DIC

A

bleeding

65
Q

chronic DIC

A

thrombotic complications

66
Q

dx of DIC

A

fibrinogen, PT, PTT, platelets, fibrin split products

67
Q

preformed IgM Abs against donor red cells

A

acute hemolytic rxn

mislabeled blood transfusion

direct coombs test positive

68
Q

fever, shaking chills, flank pain with positive direct coombs

A

acute hemolytic anemia

69
Q

delayed hemolytic reactions

A

Abs that recognize RBC antigens that recipient was sensitized to previously
-ex prior blood transfusion

typically IgG bs

positive direct coombs
low haptoglobin
elevated LDH

70
Q

low haptoglobin

A

hemolysis

71
Q

TRALI

A

transfusion related acute lung injury

factors in transfused blood product trigger activation of neutros in lung microvasculature

rare

more frequent preexisting lung conditions

72
Q

TRALI two hit hypothesis

A

first - increased sequestration and sensitization of neutros in lungs

second hit - primed neutros are activated by something in transfused blood
-Abs in transfused blood that recognizes Ags on neutros

MHC class I Ags - in multiparous women

73
Q

sudden onset of respiratory failure soon after transfusion

A

TRALI

diffuse bilateral infiltrates DO NOT respond to diuretics

also fever, hypotension, hypoxemia

67% mortality in severe cases

74
Q

bacterial transfusion infections

A

skin flora

-staph and strep

75
Q

platelet preparations

A

stored at room temp

-more likely to grow bacteria

76
Q

viral transfusion infections

A

rare - but if virus not detectable with nucleic acid testing

HIV, Hep B, Hep A

most common - is Hep B

low risk of exotic infections - west nile, trypsanosomiasis and babesiosis

HEME/LYMPH Exam 2 (16 decks)

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