Bleeding disorders Flashcards

1
Q

normal platelet count

A

150-450k

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2
Q

50-100k platelet

A

high bleeding time

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3
Q

30-50k platelet

A

bruising with minor trauma

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4
Q

10-30k platelet

A

spontaneous bruising

menorrhagia

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5
Q
A

spontaneous bruising
gum and gut bleeding
nose bleeds
brain bleeds

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6
Q

thrombocytopenia

A

low platelets

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7
Q

GP1b

A

platelet receptor for vWF binding

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8
Q

GP2b3a

A

platelet receptor for fibrinogen binding

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9
Q

ITP

A

immune thrombocytopenia purpura
IgG from splenic plasma cells against platelet antigens
splenic macrophages clear antibody covered platelets

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10
Q

symptoms of ITP

A

petechiae

ecchymosis

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11
Q

treatment of ITP

A
corticosteroids
IVIG
splectomy
TPO
rituximab
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12
Q

acute ITP

A

kids
post viral infection or immunization
self limited

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13
Q

chronic ITP

A

female

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14
Q

NAT

A

neonatal alloimmune thrombocytopenia
IgG alloantibodies (HPA-1a) from mom cross placenta and coat fetal platelets
occurs in first child- mom produces antibodies to foreign fetal platelets

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15
Q

NAT treatment

A

corticosteroids
IVIG
platelet transfusion

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16
Q

labs for ITP

A

decreased platelets
normal PT/PTT
increased megakaryocytes

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17
Q

HIT

A

heparin induced thrombocytopenia
antibodies against factor 4 produced after exposure to heparin
leads to platelet activation and thrombosis

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18
Q

type 1 HIT

A
non immune
mild thrombocytopenia
rapid onset
self resolving
asymptomatic
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19
Q

type 2 HIT

A
immune
severe thrmobocytopenia
onset 4-14d
stop heparin
thromboembolic complications
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20
Q

labs for HIT

A

platelet activation tests

HIT antibody detection

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21
Q

treatment of HIT

A

stop heparin

thrombin inhibitors

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22
Q

drug induced thrombosis

A

antibodies made against platelets after exposure to a specific drugs
lead to widespread platelet activation and thrombosis

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23
Q

TTP

A

thrombotic thrombocytopenic purport

ADAMTS13 protease inactivated by IgG leading to large aggregates of vWF that activate platelets

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24
Q

TTP pentad

A
thrombocytopenia
microangiopathic hemolytic anemia
renal dysfunction
neurologic disturbances
fever
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25
TTP thrombcytopenia
large platelets | increased megakaryocytes
26
TTP MHA
shistocytes increased LDH increased unconjugated bilirubin
27
TTP renal dysfunction
increased creatinine
28
TTP labs
normal PT and PTT
29
typical HUS
hemolytic uremic syndrome microangiopathic hemolytic anemia + thrmobicytopenia caused by enterohemorrhagic E coli Shigella-like toxins that disrupt endothelial lining leading to platelet activation in kids
30
atypical HUS
noninfective- inherited or sporadic mutations leading to excessive complement activation chronic, recurring leads to end stage renal failure
31
differentiating factor between TTP and HUS
HUS has prominent renal failure | TTP only has mildly elevated creatinine kinase
32
difference between atypical and typical HUS treatmetn
atypical treated with eculizmab
33
PFA
platelet function analyzer platelets exposed to platelet agonists and shear rate to determine inherited platelet function disorders, vWD, and meds affecting function
34
Glanzmann thrombocytopenia
GP2b/3a defect/deficiency no aggregation to ADP, collagen, or epinephrine most common inherited platelet function defect
35
bernard-souiler syndrome
GP1b defect/deficiency no aggregation to ristocetin giant platelets and mild thrombocytopenia
36
platelet against for aspirin
arachidonic acid
37
platelet against for clopidogrel (P2Y12 receptor)
ADP
38
platelet against for vWD
ristocetin
39
platelet against for bernard Soulier
ristocetin
40
routine lab tests for hemostasis
``` platelet count PT PTT fibrinogen D-dimer ```
41
specialized lab tests for hemostasis
``` bleeding time/PFA vWF testing platelet aggregation factor assays thrombin time ```
42
intrinsic pathway
PTT elevated by heparin F8, 9, 11 defieiencies
43
extrinsic pathway
PT elevated by warfarin F7 defiency
44
role of vitamin K
factors 2, 7, 9 10
45
what increases first in vitamin K deficiency and why
PT- factor 7 has shortest half life
46
coumadin reversal
FFP | prothrombin complex concentrates
47
von willenbrand disease
most common AD bleeding disorder chromosome 12 variable bleeding- mucus membranes, wounds, menorrhagia, epistaxis
48
type 1 vwd
decreased vwf levels | mild/variable bleeding
49
type 3 vwd
VERY decreased vwf levels | hemarthrosis
50
type 2 vwd
abnormal vwf that can't bind factor 8 or GP1b receptor
51
vwd labs
``` normal PTT abnormal PFA normal platelet count low ristocetin test decreased F8 activity WVF antigen activity- type 1, 3 ```
52
vwd treatment
vwf replacement DDAVP- stimulates release anti-fibrinolytic
53
hemophilia A
factor 8 deficiency | treated with factor 8
54
hemophilia B
factor 9 decency | treated with factor 9
55
PTT in hemophila
increased | corrected with mixing studies
56
factor 8 inhibitor
alloantibody developed against factor 8 in hemophilia A | treated by giving F8 or recombinant 7a
57
bethesda titer
determines strength of factor 8 inhibitor low- give 8 high- give 7a
58
DIC platelet count
decreased
59
DIC D-dimer
increase
60
DIC PT
increase
61
DIC PTT
increase
62
DIC thrombin time
increase
63
DIC fibrinogen
decrease