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Flashcards in Bleeding Disorders Deck (40):
1

How do you test primary hemostasis? (3)

1. Platelet Count
2. Bleeding Time
3. Platelet Function Analysis (PFA) ***

2

How do you test secondary hemostasis? (4)

1. PTT (Partial Thromboplastin Time
2. PT (Prothrombin Time)
3. Mixing Study
4. Clotting Factors

3

PTT tests what?

Intrinsic and common pathway
12, 11, 9, 8

4

PT tests what?

Extrinsic and common pathway
7

5

Scurvy

Vitamin C Deficient

6

Ehlers-Danlos Syndrome

inherited collagen abnormality

7

Cushing Syndrome

Excessive Steroid = protein wasting = loss of vascular support

8

Henoch-Schonlein Purpura

Systemic Hypersensitivity disease in which circulating immune complexes deposit in vessels

9

Hereditary Hemorrhagic Telandiectasia

AD; dilated tortuous vessels with thin walls

10

Chronic Immune Thrombocytopenic Purpura (ITP)

Adults
IgG against platelet antigens
Giant platelets (HIGH MPV)
Normal Spleen
Increased number Megakaryocytes (LEFT SHIFTED)
Steroids or Rituximab

11

Acute ITP

Kids
Follows viral infection

12

Heparin Induced Thrombocytopenia (HIT) Type 1

MOST COMMON
Direct platelet aggregation due to heparin
Within a few days of drug
Moderate thrombocytopenia
Clinically insignificant

13

HIT 2

5-14 days after heparin
Mod to Severe Thrombocytopenia
Abs against heparin-platelet-factor 4 complex resulting in direct platelet activation
PARADOXICAL THROMBOSIS (making clots)
LIFE THREATENING

HIT Lovenox (low mw) also contraindicated

14

HIV Associated Thrombocytopenia

Common
CD4
Direct infx of megakaryocytes by HIV
Dysregulation and hyperplasia of B cells
auto AB towards lately Gp2b3a

15

TTP Pentad

1. Fever
2. Thrombocytopenia
3. Microangiopathic Hemolytic Anemia (damaged RBCs)
4. NEUROLOGIC SX
5. Renal Failure

16

TTP Cause

ADAMTS 13 Deficiency -- accumulation of vWF multimers bc they are not being broken down

Acquired - auto AB against Adams
Inherited - inactivated gene mutation

17

TTP Treatment

Total Plasma Exchange

PLATELET TRANSFUSION = CONTRAINDICATED -- worsens the thrombi

18

HUS

Bloody Diarrhea
RENAL FAILURE
E. Coli 0157 H7
Kids

19

Bernard-Soulier

AR
No Gp1b
Giant Platelets
Treat: Everything works BUT ristocetin

20

Glanzmann's Thrombasthenia

AR
No Gp2b3a
Treat: ONLY Ristocetin

21

von Willebrand Disease

Most common bleeding disorder
Prolonged bleeding (MUCOUS MEMBRANES)
Abnormal PFA and PTT
Normal Platelet count
AD

22

von Willebrand type 1 (70%)

Quantitative decrease in vWF
Usually no treatment ...DDAVP can be given before a procedure

23

von Willebrand type 2 (25%)

Qualitative vWF abnormality
2A is most common
Defective multimer assembly

24

von Willebrand type 3 (rare)

quantitative decrease
AR

25

Hemophilia A (8 deficient)

X linked Recessive
Males and Homozyg Females
30% with no family history
Easy bruising, Massive hemorrhage after surgery

HEMARTHROSES = spontaneous hemorrhage into large joints

Diagnose = Prolonged PTT
Treat= Humate P

26

Hemophilia B/Christmas (9)

Identical to 8 bc both work together to activate 10
X - R
Some have normal levels but abnormal function

Diag = prolonged PTT
Treat = recombinant Factor 9

27

Acquired Clotting Factor Deficiencies (3)

1. Vit K Deficient (Coumadin)
2. Liver Disease (where coag factors are made)
3. DIC

28

DIC (def)

Physiologic consequence of other diseases:
1. Thrombohemorrhagic Disorder
2. Activation of cascade in microvasculature
3. Tissue Hypoxia/infarct

29

DIC Diagnose

1. Prolonged PT, PTT
2. Decreased Platelet
3. Decreasing Fibrinogen
4. Increased D-Dimer
5. Peripheral smear: shistocytes; giant platelets

30

Causes of DIC

1. Placental Abruption
2. APl
3. Adenocarcinoma
4. Massive Tissue Injury

31

Mechanisms of DIC

1. Tissue Factor release into blood (APL)
2. Wide spread endothelial cell injury (infectious etiologies via TNF production)

32

Consequences of DIC

1. Widespread fibrin deposition in microvasculature
- ischemia of associated organs
- hemolytic anemia
2. Hemorrhagic Diathesis
- factor and platelet consumption faster than production
- plasminogen activation

33

Morphology of DIC

Thrombi in small vessels
CNS Micro infarts/hemorrhage
Waterhouse-Friderichsen Syndrome (massive bilateral adrenal hemorrhage associated with meningococcus)
Sheehan Syndrome
Toxemia of Pregnancy

34

Clinical Course of DIC

Acute -- bleeding
Chronic -- thrombosis

35

Treatment of DIC

Treat underlying issue
Supportive care

36

Overview of Vessel Wall Abnormalities

Petechiae and Purpura
Normal Coag Tests

37

Overview of Thrombocytopenia

Decreased Platelet count
BT and PFA prolonged
Normal PT and PTT

38

Post-Traumatic Bleeding

20,000 - 50,000 platelet count

39

Spontaneous bleeding of small vessels

40

Platelet Function Abnormalities (Overview)

PT, PTT, Platelet count NORMAL
PFA - abnormal