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
hepatic failure
lost coag factors | -NOT VIII and vWF
26
vWD vs. Hem A
vWD - epistaxis (nosebleeds), bruising, petechiae, GI bleeds, mennorrhagia Hem A - hematoma, hemarthrosis
27
ristocetin test
check for vWF: GpIb interaction | -check for bernard soulier**
28
ADP/collagen/epinephrine/arachidonic acid test
check for GpIIbIIIa : fibrinogen (or vWF) binding | -check for glanzsmann thrombasthenia
29
gray platelet syndrome
empty platelet alpha granules result sin mild bleeding auto dom or rec thrombocytopenia, myelofibrosis, hypogranular platelets, giant platelets
30
graph of platelet count vs. bleeding time
down to left - decreased platelet number but increased activity -ITP up to right - decreased platelet function -aspirin, uremia, vWD
31
ITP
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
thrombotic microangiopathies
TMA - thrombocytopenia and hemolytic anemia - neuro symptoms, fever, renal dysfunction - normal PT and aPTT
33
TTP
thrombotic thrombocytopenic purpura | -deficient ADAMTS13 (which degrades large amounts of vWF)
34
STEC-HUS
shiga toxin E-Coli hemolitic uremic syndrome E. coli 0157:H7 bloody diarrhea
35
HELLP syndrome
hemolysisn - elevated liver enzymes - low platelet - pregnant women with HTN and proteinuria
36
DIC
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
waterhouse-friedrichsen syndrome
microthrombi to arenal cortex - massive adrenal hemorrhage with DIC
38
sheehan postpartum necrosis
microthrombi to pituitary gland with DIC
39
Tx of DIC
important to treat the cause
40
serum
has no coag factors red tube
41
plasma
add anticoagulant - has coag factors add sodium citrate / EDTA blue tube - sodium citrate lavender tube - EDTA
42
haemophilia
prolonged aPTT
43
aspirin
decreased platelet function
44
vit K deficiency
or warfarin prolonged PT and aPTT
45
DIC
long PT and aPTT | decreased platelets
46
vWD
prolonged aPTT and decreased platelet function
47
early liver failure
PT prolonged
48
end stage liver failure
long PT and aPTT | -decreased platelet function and count
49
uremia
decreased platelet function
50
factor V deficiency
long PT and aPTT
51
bernard soulier
decreased platelet count and function
52
glanzmann
decreased platelet function