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

RBC and Bleeding Disorders VII Flashcards

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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
inital binding of vWE to endothelial
GpIb
26
bridging of fibrinogen in platelet aggregation
GpIIb/IIIa
27
most common inherited bleeding disorder of humans
vWD mild bleeding tendency - surgery or dental procedure spontaneuous epistaxis auto domimant
28
three categories of vWD
type 1 and 3 - quantitative | type 2 - qualitative
29
type I vWD
auto dominant -quantitative defect 70% of cases mild disease point substitutions -rapid clearance from plasma
30
type III vWD
auto recessive -quantitative defect severe clinically - very low vWF effects factor VIII stability deletions or frameshift mutations of both alleles
31
type II vWD
qualitative defect -2A - most common form auto dominant missense mutations - defective multimer assembly 25% of cases mild to moderate bleeding
32
vWD clinical
prolonged PTT - due to lack of factor VIII stability | reduced vWF - measured by ristocetin
33
desmopressin
tx of vWD patient undergoing surgery or dental work causes vWF release
34
most common hereditary disease associated with life-threatening bleeding
Hemophilia A mutation in factor VIII X-linked recessive affects mainly males
35
hem A
mutation in factor VIII X-linked recessive affects mainly males rarely in females - inactivation- unfavorable lyonization
36
<1% Factor VIII
severe Hem A
37
2-5% factor VIII
moderately severe Hem A
38
>6% factor VIII
mild disease Hem A
39
most severe deficiency of factor VIII
involve inversion involving X-chromosome that completely abolishes the synthesis of factor VIII
40
easy bruising, massive hemorrhage after trauma, hemarthroses, NO petechiae
hem A
41
prolonged PTT and normal PT
hem A defect in intrinsic pathway
42
diagnosis of Hem A
factor VIII specific assays
43
Tx Hem 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
christmas disease
Hem B factor IX deficiency clinically indistinguishable from Hem A X-linked recessive PTT prolonged and PT normal
45
diagnosis of christmas disease
only possibly by assay of factor levels
46
tx of Christmas disease
infusion of recombinant factor IX
47
hypoxia and infarction as well as hemorrhage
DIC deposition of microthrombi as well as depletion of heme factors
48
DIC
not primary disease coagulopathy occuring in course of variety of clinical condition
49
activation of factor X
generation of thrombin thrombin converts fibrinogen to fibrin
50
thrombomodulin
binds and inactivates thrombin this complex binds and activates protein C -inhibitor of factors V and VIII
51
mechanisms triggering DIC
widespread injury to endo cells release of tissue factor
52
mucus from adenocarcinomas
procoagulant - can initiate DIC
53
TNF
induces endo cells to express tissue factor - can lead to sepsis
54
causes of CID
most likely obstetric complications malignant neoplasms sepsis major trauma
55
bacterial infection
endotoxins inhibit expression of thrombomondulin | -to DIC
56
massive trauma
release of tissue factor | -to DIC
57
consequences of DIC
deposition of fibrin within microcirculation -can lead to ischemia and microangiopathic hemolytic anemia consumption of platelets and clotting factors - hemorrhagic diathesis
58
plasmin
cleaves fibrin and digests factor V and VIII
59
bilateral renal cortical necrosis
imcroinfarcts from DIC
60
waterhouse friedrishsen syndrome
massive adrenal hemorrhage | -with DIC from meningococcemia infection
61
kasabasch merritt syndrome
form of DIC with giant hemangiomas thrombin form with neoplasm
62
DIC clinical
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
microangiopathic hemolytic anemia, dyspnea, cyanosis, resp failure, convulsions coma, oliguria, acute renal failure, circ failure and shock
DIC
64
acute DIC
bleeding
65
chronic DIC
thrombotic complications
66
dx of DIC
fibrinogen, PT, PTT, platelets, fibrin split products
67
preformed IgM Abs against donor red cells
acute hemolytic rxn mislabeled blood transfusion direct coombs test positive
68
fever, shaking chills, flank pain with positive direct coombs
acute hemolytic anemia
69
delayed hemolytic reactions
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
low haptoglobin
hemolysis
71
TRALI
transfusion related acute lung injury factors in transfused blood product trigger activation of neutros in lung microvasculature rare more frequent preexisting lung conditions
72
TRALI two hit hypothesis
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
sudden onset of respiratory failure soon after transfusion
TRALI diffuse bilateral infiltrates DO NOT respond to diuretics also fever, hypotension, hypoxemia 67% mortality in severe cases
74
bacterial transfusion infections
skin flora | -staph and strep
75
platelet preparations
stored at room temp | -more likely to grow bacteria
76
viral transfusion infections
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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