Bleeding disorders Flashcards Preview

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Flashcards in Bleeding disorders Deck (44):
1

Bleeding that is:
-superficial
-petechiae
-spontaneous

Platelet bleeding

2

Bleeding that is:
-deep joints
-Big bleeds
-Trauma

Factor bleeding

3

Most common hereditary bleeding disorder

Autosomal dominant

Variable severity

Von Willebrand Disease

**vW factor decreased or abnormal

4

vW factor made where?

megakaryocytes and subendothelial cells

5

vWF carries what factor?

VIII

6

Three types of vW disease:

Type 1 (70%) : decreased vWF

Type 2 (25%) : abnormal vWF

Type 3 (5%) : no vWF

7

Sx of vW disease?

mucosal bleeding

deep joint bleeding (severe cases)

8

What test will be NORMAL in a pt with vW disease?

INR

9

Platelet membrane protein that binds vWF?

GP Ib

10

Tx fro vW disease?

DDAVP (for type I)

Cryoprecipitate (contains vWF and VIII)

Factor VIII

11

X-linked recessive bleeding disorders:

hemophilia A and B

12

most common FACTOR deficiency

factor VIII decreased

hemophilia A

13

common sx of hemophilia A:

deep joint bleeding

prolonged bleeding after dental work

14

NORMAL lab results in hemophilia A and B?

INR

TT

Platelet count

bleeding time

15

What does PTT look like in hemophilia?

prolonged (low factor VIII or IX)

**corrects with mixing study

16

Tx of hemophilia A:

DDAVP

Factor VIII

17

What is the difference between hemophilia A and B

A = VIII problem

B = IX problem, MUCH LESS COMMON

18

Rare; bleeding only after trauma:

XI deficiency

19

severe neonatal bleeding

XIII

20

Crosslinks fibrin --> clot

XIIIa

21

Abnormal GP Ib

Big platelets

Severe Bleeding

Bernard-Soulier Syndrome

22

No IIb-IIIa

No aggregation

Severe bleeding

Glanzmann Thrombasthenia

23

No alpha granules in platelets

Big, empty platelets

Mild bleeding

Gray Platelet Syndrome

24

Platelet prob related to Chediak-Higashi?

delta Granule deficiency

25

Lots of causes

coag triggered causing thrombosis

platelets and factors get used up, causing bleeding

MAHA

DIC

**Disseminated Intravascular Coagulation

26

DIC triggers that "dump" coag factors causing DIC:

OB complications

Adenocarcinoma

AML (promyelocytic)

27

DIC triggers that "rip" endothelial cells apart causing DIC:

Bacterial sepsis

Trauma

Burns

Vasculitis

28

Top 4 causes of DIC*****:

Malignancy

OB complications

Sepsis

Trauma

29

INR, PTT, TT in DIC?

prolonged

30

FDP's in DIC?

increased

31

Fibrinogen in DIC?

decreased

32

Antiplatelet antibodies

Acute vs Chronic

Dx of exclusion

Tx steroids or splenectomy

Ideopathic Thrombocytopenic Purpura (ITP)

33

What do the Igs bind to in ITP?

GP IIb-IIIa or Ib

***get eaten by splenic mac's

34

Demographic for chronic ITP?

adult women

35

Demographic for acute ITP?

kids

36

ADAMTS13 deficiency

Thrombotic Thrombocytopenic Purpura

37

MAHA, ____________, fever, neurologic defects, renal failure

Big vWF multimers trap platelets

TTP

Thrombotic Thrombocytpenic Purpura

38

Multimers of Unusual Size

TTP

39

Clinical findings of:

Hematuria, jaundice (MAHA)

bleeding, bruising

Fever

Bizarre behavior (NEUROLOGICAL DEFICITS)

Decreased urine output (RENAL FAILURE)

TTP

40

MAHA, Thrombocytopenia

E. coli*******

Toxin or damage to endothelium

Hemolytic Uremic Syndrome

41

Defect in complement factor H

Non-epidemic (E. coli related) Hemolytic Uremic Syndrome

42

Brain involvement think?

TTP

43

Kidney involvement think?

HUS

44

contraindicated tx in HUS?

Abx!!!!

may increase toxin release