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
Q

TTP thrombcytopenia

A

large platelets

increased megakaryocytes

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26
Q

TTP MHA

A

shistocytes
increased LDH
increased unconjugated bilirubin

27
Q

TTP renal dysfunction

A

increased creatinine

28
Q

TTP labs

A

normal PT and PTT

29
Q

typical HUS

A

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
Q

atypical HUS

A

noninfective- inherited or sporadic mutations leading to excessive complement activation
chronic, recurring
leads to end stage renal failure

31
Q

differentiating factor between TTP and HUS

A

HUS has prominent renal failure

TTP only has mildly elevated creatinine kinase

32
Q

difference between atypical and typical HUS treatmetn

A

atypical treated with eculizmab

33
Q

PFA

A

platelet function analyzer
platelets exposed to platelet agonists and shear rate to determine inherited platelet function disorders, vWD, and meds affecting function

34
Q

Glanzmann thrombocytopenia

A

GP2b/3a defect/deficiency
no aggregation to ADP, collagen, or epinephrine
most common inherited platelet function defect

35
Q

bernard-souiler syndrome

A

GP1b defect/deficiency
no aggregation to ristocetin
giant platelets and mild thrombocytopenia

36
Q

platelet against for aspirin

A

arachidonic acid

37
Q

platelet against for clopidogrel (P2Y12 receptor)

A

ADP

38
Q

platelet against for vWD

A

ristocetin

39
Q

platelet against for bernard Soulier

A

ristocetin

40
Q

routine lab tests for hemostasis

A
platelet count
PT
PTT
fibrinogen
D-dimer
41
Q

specialized lab tests for hemostasis

A
bleeding time/PFA
vWF testing
platelet aggregation
factor assays
thrombin time
42
Q

intrinsic pathway

A

PTT
elevated by heparin
F8, 9, 11 defieiencies

43
Q

extrinsic pathway

A

PT
elevated by warfarin
F7 defiency

44
Q

role of vitamin K

A

factors 2, 7, 9 10

45
Q

what increases first in vitamin K deficiency and why

A

PT- factor 7 has shortest half life

46
Q

coumadin reversal

A

FFP

prothrombin complex concentrates

47
Q

von willenbrand disease

A

most common AD bleeding disorder
chromosome 12
variable bleeding- mucus membranes, wounds, menorrhagia, epistaxis

48
Q

type 1 vwd

A

decreased vwf levels

mild/variable bleeding

49
Q

type 3 vwd

A

VERY decreased vwf levels

hemarthrosis

50
Q

type 2 vwd

A

abnormal vwf that can’t bind factor 8 or GP1b receptor

51
Q

vwd labs

A
normal PTT
abnormal PFA
normal platelet count
low ristocetin test
decreased F8 activity
WVF antigen activity- type 1, 3
52
Q

vwd treatment

A

vwf replacement
DDAVP- stimulates release
anti-fibrinolytic

53
Q

hemophilia A

A

factor 8 deficiency

treated with factor 8

54
Q

hemophilia B

A

factor 9 decency

treated with factor 9

55
Q

PTT in hemophila

A

increased

corrected with mixing studies

56
Q

factor 8 inhibitor

A

alloantibody developed against factor 8 in hemophilia A

treated by giving F8 or recombinant 7a

57
Q

bethesda titer

A

determines strength of factor 8 inhibitor
low- give 8
high- give 7a

58
Q

DIC platelet count

A

decreased

59
Q

DIC D-dimer

A

increase

60
Q

DIC PT

A

increase

61
Q

DIC PTT

A

increase

62
Q

DIC thrombin time

A

increase

63
Q

DIC fibrinogen

A

decrease