Coagulophathies

Question 1

Coagulopathy categories?

Answer 1

Clotting
Platelet
Hypercoag

Question 2

Clotting disorders? (2)

Answer 2

Hemophilia A (factor 8)
Hemo B (factor 9)

Question 3

Petechiae?

Answer 3

< 2mm
Focal SQ bleed
Don’t blanch

Question 4

Purpura?

Answer 4

2-10mm
Palpable
Don’t blanch

Question 5

Ecchymoses?

Answer 5

> 1cm

Question 6

Initial labs for bleeding disorder? (4)

Answer 6

CBC w/ platelet count
Periphial smear
PT/INR, aPTT
+/- bleed time

Question 7

Platelets levels:

thrombocytopenia?

prolonged bleed from injury?

spontaneous bleed?

Answer 7

< 100k

40k

<20k

Question 8

Bleed time measures?

Screens for?

Prolonged if?

Answer 8

Time for hemostasis

Microvasculature and platelet fxn

Platelet disorder

Question 9

Hemostatic response to vessel injury?

Answer 9

1st vasospasm
2nd platelet aggregation
3rd coag cascade

Question 10

Extrinsic Pathway includes what Factors?

Requires what for activation?

What test measure Extrinsic path?

What med interrupts Extrinsic path?

Answer 10

Factor VII

Tissue Factor

PT (also measures Common)

Warfarin

Question 11

Intrinsic Pathway includes what Factors?

Requires what for activation?

What test measures Intrinsic path?

What med interrupts Intrinsic path?

Answer 11

VIII, IX, XI, XII

Injury

PTT (also measures Common)

Heparin

Question 12

Common Pathway includes what Factors?

What test measures Common path?

What med interrupts Common path?

Answer 12

I (fibrinogen), II (prothromin), V, X

PTT and PT

Lovanox

Question 13

Prothrombin Time (PT) monitors what Path?

What meds?

What conditions? (2)

Answer 13

Extrinsic path

Warfarin

Liver fxn/damage
DIC

Question 14

Partial Thromboplastin Time (PTT) or
Activate Partial (aPTT) monitor what Path?

What meds?

What conditions? (5)

Answer 14

Intrinsic

Unfractionated Heparin (NOT LMWH)

Hemophilia, vW, liver damage, Vit K def, DIC

Question 15

International Normalized Ratio (INR) monitors what?

Answer 15

Same as PT but more specific measures dependent on condition

Question 16

INR target ranges:

DVT prophy?

DVT (acute), VTE, AFib?

Heart valves?

Answer 16

1.5 - 2

2 -3

2.5 - 3.5

Question 17

PTT mixing study tests what?

Answer 17

Coag factor deficiency vs inhibitor problem

Mix pt blood w/ blood w/ normal factors,
If corrects, then pt missing factors
If not, pt has inhibitor problem

Question 18

Thrombin Time (TT) tests what?

Answer 18

Fibrinogen

Question 19

Clotting Disorders are?

Answer 19

Congenital or acquired

Excess or repetitive bleed at UNUSUAL sites w/ normal activity

U joints, mm, GI, GU

Question 20

Hemophilia A/B details?

Answer 20

A = VIII (most common)
B = IX (Christmas)

X-linked recessive
M&raquo_space; F

Question 21

Hemophilia A/B presentation?

Answer 21

Asympt until 1st bleed U by 2yo

Severe pain in weight-bearing joints

Question 22

Hemo A tests?

Answer 22

Platelet count = N
PT = N
aPTT = long but corrects w/ PTT mix study
Factor VIII assay

Question 23

Platelet Disorders: Low

Questions? (5)

Answer 23

Sustained ↓?
Recent infection?
Meds that ↓ platelets?
Bleed/Not bleed OFP to platelets?
Evidence of:
-platelet consumption synd?
-other autoimm?
-chronic liver dz?

Question 24

Platelet Disorders: High

Questions? (6)

Answer 24

Cause of significant inflamm?
Current infection?
Peripheral smear abnormal?
Fe def anemia?
Inapprop clotting/bleeding?
Signs of:
-essential thrombocytosis?
-CA

Question 25

Platelet Disorders: Dysfxn Caused by? (2)

Answer 25

Congenital (rare) | Acquired (drugs/EtOH, uremia, myelopro dz)

Question 26

Platelet Disorders: Dysfxn Lab tests?

Answer 26

Count = N Morphology = N Bleed Time = long

Question 27

Platelet Disorders: Splenic Sequestration?

Answer 27

``` Hyper/Megaly = cytopenia Ectomy = cytosis ```

Question 28

Idiopathic Thrombocytopenic Purpura (ITP) result of? (3) Acute? Chronic?

Answer 28

Platelet destruction ↓ prodxn Sequestration A = U kids post URI, Heparin (most C inpt cause) C = a/w autoimm, HIV, 20-50yo

Question 29

ITP presentation? (3)

Answer 29

Petechia, purpura, bullae on skin/muc memb Epistaxis, oral bleeding Subarachnoid/Intracranial hemorrhage

Question 30

ITP labs? (2)

Answer 30

``` Coag studies = N Platelet count = ↓↓ ```

Question 31

ITP tx? (4)

Answer 31

No NSAIDs/ASA Prednisone IV IG Platelet transfusion

Question 32

DIC (Disseminated Intravascular Coag) is? a/w?

Answer 32

Widespread coag -> massive platelet, fibrin, factor consumption -> uncontrolled bleeding underlying systemic illness

Question 33

Illness a/w DIC? (4)

Answer 33

Sepsis Trauma CA Preggo complications ``` MOST ????? Malaria Osteomyelitis Sepsis Trauma ```

Question 34

DIC presentation?

Answer 34

Variable onset Multi organ systems Hemorr

Question 35

DIC labs? (6)

Answer 35

``` Platelets = ↓ Fibrinogen = ↓ Fibrinolysis = ↑ PT/PTT = ↑ D-dimer = ↑ Smear = schistocytes ```

Question 36

DIC tx? (4)

Answer 36

``` Transfusion w/: Cryoprecipitate (fibrinogen replacement) FFP (coag factors) Platelets PRBCs ```

Question 37

Thrombotic Thrompocytopenia Purpura (TTP) is? Caused by? (4)

Answer 37

Rare, U fatal Microangiopathic hemolytic anemia + thrombocytopenia -> endothelial injury/small vessel thrombi E. coli O157:H7 CA Preggo Drugs

Question 38

TTP common in who?

Answer 38

20-50yo F>M +HIV

Question 39

TTP presentation? (6)

Answer 39

``` Toxic Abd pain HA Mental ∆s Pallor, petechiae Jaundice ```

Question 40

Hemolytic Uremic Synd is? Presentation? Labs? (4) Tx?

Answer 40

U from e. coli O157:H7 Abd pain w/ bloody D days prior Like TTP less CNS, more renal Hemolytic anemia Thrombocytopenia Schistocytes Renal fail Adults: plasmapheresis Kids: IV hydration w/ e-

Question 41

TTP-HUS labs?

Answer 41

``` Microangiopathic hemolytic anemia Thrombocytopenia Smear = Schistocytes Coag = N (diff from DIC) Renal insuff ```

Question 42

TTP-HUS tx? (4)

Answer 42

Steroids Plasmapheresis (plasma exchange) Rituximab if refractory High mortality

Question 43

SUMMARY: TTP triad?

Answer 43

Hemolytic anemia Thrombocytopenia Neuro deficits (ADAMTS13 mutation)

Question 44

SUMMARY: HUS triad?

Answer 44

Hemolytic anemia Thrombocytopenia Renal insuff

Question 45

Von Willebrand's Dz epidemiology?

Answer 45

Auto dominant M=F Most C coag

Question 46

vWF causes? Type I?

Answer 46

↓ Factor VIII ANTIGEN -> deficient or defective vWF Most C Acquired (bone marrow CA, valproic acid, SLE) ↓ Factor VIII

Question 47

vWF presentation? (4)

Answer 47

Bleed (nose, vag, GI) Hemarthrosis/Soft tissue bleed less likely Bleed ↑ w/ ASA Bleed ↓ E2/preggo

Question 48

vWF labs?

Answer 48

Bleed time = long (N in hemophilia) INR = N vWF = low

Question 49

vWF tx?

Answer 49

Desmopressin (releases stores vWF from endo cells) Factor VIII infustion Humate-P (VIII and vWF)

Question 50

Hypercoag States: Congenital?

Answer 50

U auto-dominant Deficiencies in: Antithombin (III) Prot C or S Mutations in: Factor V (Leiden) Prothrombin (II)

Question 51

Antithrombin III deficiency epidemiology? Causes what? What med acts on III?

Answer 51

Congenital or acquired (DIC, HUS) Thromboembolic dz Lack of III means no inhibition of coag cascade Heparin (↑ effects of III)

Question 52

Antithrombin III Deficiency presentation? Labs? Tx? (2)

Answer 52

FHx of DVT, PE U 20-30yo U asympt Acute thrombosis or embolism III = low IV heparin Long-term Warfarin

Question 53

Protein C Deficiency epidemiology? Causes what? Presentation?

Answer 53

Auto dominant Prot C is Vit-K dependent DVTs Lack of C means no fibrinolysis ∴ no clot lysis FHx Spontaneous or provoked DVT

Question 54

Protein C Deficiency labs? Tx? (3)

Answer 54

Prot C = low IV heparin Long-term Warfarin No oral contraceptives or hormone replacement

Question 55

Protein S Deficiency epidemiology? Causes what? Presentation?

Answer 55

Auto domin or Acquired (liver dz, vit K def) VTE, thromboembol Lack of S means no Prot C activation VTE U <40yo Spontaneous abortion

Question 56

Protein S Deficiency labs? Tx?

Answer 56

Prot S antigen = low Same as Prot C + prophy w/ preggos

Question 57

Factor V Leiden Mutation epidemiology? Causes what?

Answer 57

Hereditary thrombophilia Resists activated Prot C -> venous thrombosis, thromboembolism

Question 58

Factor V Leiden Mutation labs: aPTT PTT

Answer 58

aPTT = screen for activated Prot C (APC) resistance PTT = measure w/ APC and w/o APC, If resistance +, PTTs will be equal If resistance -, PTT longer w/ APC If abnormal, test for mutation

Question 59

Factor V Leiden Mutation tx?

Answer 59

same