Bleeding disorders Flashcards Preview

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Flashcards in Bleeding disorders Deck (63):
1

normal platelet count

150-450k

2

50-100k platelet

high bleeding time

3

30-50k platelet

bruising with minor trauma

4

10-30k platelet

spontaneous bruising
menorrhagia

5

spontaneous bruising
gum and gut bleeding
nose bleeds
brain bleeds

6

thrombocytopenia

low platelets

7

GP1b

platelet receptor for vWF binding

8

GP2b3a

platelet receptor for fibrinogen binding

9

ITP

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

10

symptoms of ITP

petechiae
ecchymosis

11

treatment of ITP

corticosteroids
IVIG
splectomy
TPO
rituximab

12

acute ITP

kids
post viral infection or immunization
self limited

13

chronic ITP

female

14

NAT

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

15

NAT treatment

corticosteroids
IVIG
platelet transfusion

16

labs for ITP

decreased platelets
normal PT/PTT
increased megakaryocytes

17

HIT

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

18

type 1 HIT

non immune
mild thrombocytopenia
rapid onset
self resolving
asymptomatic

19

type 2 HIT

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

20

labs for HIT

platelet activation tests
HIT antibody detection

21

treatment of HIT

stop heparin
thrombin inhibitors

22

drug induced thrombosis

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

23

TTP

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

24

TTP pentad

thrombocytopenia
microangiopathic hemolytic anemia
renal dysfunction
neurologic disturbances
fever

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