Thrombosis III Flashcards

1
Q

bernard soulier syndrome

A

Gp1b deficiency

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2
Q

von willebrand disease

A

vWF deficiency

factor VIII - complexed with vWF - so don’t get that either

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3
Q

glanzmann thrombasthenia

A

GpIIb/IIIa deficiency

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4
Q

essential thrombocythemia

A

lots of platelets - but they are dysfunctional

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5
Q

amyloidosis

A

light chain deposition weakens walls

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6
Q

hemophilia A

A

factor VIII deficiency

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7
Q

hemophilia B

A

factor IX deficiency

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8
Q

hemophilia C

A

factor XI deficiency

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9
Q

mucocutaneous bleeding

A

likely problem with primary hemostasis

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10
Q

spontaneous bleeding of soft tissues, muscles, joints, delayed surgical bleeding

A

likely problem of coag factors

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11
Q

type I von willebrands disease

A

autosomal dominant

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12
Q

chediak higashi syndrome

A

albinism

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13
Q

digeorge

A

CATCH 22

cleft palate, cardiac defect, facial abnormalities, learning disability

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14
Q

euro jews and basque spain/s. france

A

hemophilia C

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15
Q

jaundice with coag factor deficiency

A

liver failure

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16
Q

sex linked

A

hem A and B

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17
Q

petechiae

A

< 0.3 cm hemorrhage

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18
Q

purpura

A

0.3 - 1 cm hemorrhage

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19
Q

ecchymoses

A

> 1 cm hemorrhage

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20
Q

factor II

A

mucosal and deep tissue bleeding

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21
Q

quantitative vWD

A

type 1 and 3
-reduced quantity of vWF

1 - mild deficiency
3 - severe deficiency (with decreased factor VIII)

22
Q

qualitative vWD

A

type 2

-dysfunctional vWF

23
Q

christmas disease

A

factor IX deficiency - hem B

24
Q

washout

A

bleeding disorder from washing out all coag factors

-ex. IV fluid admin

25
Q

hepatic failure

A

lost coag factors

-NOT VIII and vWF

26
Q

vWD vs. Hem A

A

vWD - epistaxis (nosebleeds), bruising, petechiae, GI bleeds, mennorrhagia

Hem A - hematoma, hemarthrosis

27
Q

ristocetin test

A

check for vWF: GpIb interaction

-check for bernard soulier**

28
Q

ADP/collagen/epinephrine/arachidonic acid test

A

check for GpIIbIIIa : fibrinogen (or vWF) binding

-check for glanzsmann thrombasthenia

29
Q

gray platelet syndrome

A

empty platelet alpha granules

result sin mild bleeding

auto dom or rec

thrombocytopenia, myelofibrosis, hypogranular platelets, giant platelets

30
Q

graph of platelet count vs. bleeding time

A

down to left - decreased platelet number but increased activity
-ITP

up to right - decreased platelet function
-aspirin, uremia, vWD

31
Q

ITP

A

child immune (idiopathic) thrombocytopenia purpura

  • autoantibodies against platelet antigens
  • childhood disease with acute onset
  • goes away

adult chronic immune (refractory) thrombocytopenia purpura

  • autoantibodies against platelet antigens
  • adults - 20-40yo, female, history of infection, does not go away
32
Q

thrombotic microangiopathies

A

TMA

  • thrombocytopenia and hemolytic anemia
  • neuro symptoms, fever, renal dysfunction
  • normal PT and aPTT
33
Q

TTP

A

thrombotic thrombocytopenic purpura

-deficient ADAMTS13 (which degrades large amounts of vWF)

34
Q

STEC-HUS

A

shiga toxin E-Coli hemolitic uremic syndrome

E. coli 0157:H7

bloody diarrhea

35
Q

HELLP syndrome

A

hemolysisn

  • elevated liver enzymes
  • low platelet
  • pregnant women with HTN and proteinuria
36
Q

DIC

A

all clotting steps occuring at same time everywhere

consumptive coagulopathy with bleeding

causes:

  • infection
  • trauma
  • malignancy
  • toxins
  • liver disease
  • prosthetic device
  • autoimmunity
  • vascular disorders

microthrombi ofrm in many tissues

37
Q

waterhouse-friedrichsen syndrome

A

microthrombi to arenal cortex
- massive adrenal hemorrhage

with DIC

38
Q

sheehan postpartum necrosis

A

microthrombi to pituitary gland

with DIC

39
Q

Tx of DIC

A

important to treat the cause

40
Q

serum

A

has no coag factors

red tube

41
Q

plasma

A

add anticoagulant - has coag factors

add sodium citrate / EDTA

blue tube - sodium citrate
lavender tube - EDTA

42
Q

haemophilia

A

prolonged aPTT

43
Q

aspirin

A

decreased platelet function

44
Q

vit K deficiency

A

or warfarin

prolonged PT and aPTT

45
Q

DIC

A

long PT and aPTT

decreased platelets

46
Q

vWD

A

prolonged aPTT and decreased platelet function

47
Q

early liver failure

A

PT prolonged

48
Q

end stage liver failure

A

long PT and aPTT

-decreased platelet function and count

49
Q

uremia

A

decreased platelet function

50
Q

factor V deficiency

A

long PT and aPTT

51
Q

bernard soulier

A

decreased platelet count and function

52
Q

glanzmann

A

decreased platelet function