Acquired Coagulation Disorders

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1.

Acute ITP

-children
-self limited
-after immunization or virus

2.

ADAMS TS13

-degrades vWF (breaks up the multimer)

-if deficient: vWF piles up, causes abnormal
platelet adhesion

3.

Alpha 2
Antiplasmin

-shuts down plasmin (inactivates)

4.

Aspirin

(primary, qualitative)
-inactivates cyclooxygenase, lack of TXA2
-impairs platelet aggregation

5.

Bernard Soulier
Syndrome

13.

Disseminated
Intravascular
Coagulation

(DIC)
-depletion of platelets and coag factors >bleeding
-deposition of fibrin -> microvessel
thombosis

14.

Examples of
Fibrinolysis
Disorders

-radical prostatectomy: urokinase

released, activates plasmin
-cirrhosis of liver: reduced alpha 2
antiplasmin

15.

Fibrinolysis
Disorders

-overactive plasmin -> too much

fibrinogen cleavage
-last stage of coagulation, to remove
thrombus
-because they are due to pathology,
theres no thrombus to remove, s plasmin
acts ands destroys coag factors

16.

Glanzmann
thrombasthenia

(primary, qualitative)
-genetic def in gp1b
-mild thrombocytopenia
-LARGE platelets (Big Suckers)
6.

7.

8.

Chronic ITP

Coagulation
Factor Inhibitor

Conditions
Associated
w/DIC

-women
-primary or secondary
-can pass IgG to offspring

(primary, qualitative)
-genetic gp2b/3a def
-platelet aggregation imparied

(secondary hemostatic disorder)

-AQUIRED Ab against coag factor ->
impaired function
-F8 most common
-same symptoms and labs as hemophilia A

17.

Hemophilia A

-sepsis
-trauma: head injury, tissue injury, fat emboli
-obstetrical complications: amniotic fluid
embolus, abruption, retained dead fetus
-Cancer: Trousseau syndrome
-Immune disorders
-toxins
-vascular disorders

(secondary hemostatic disorder)

-xlinked recessive
-factor 8 def
-elevated PTT, low PT
-low factor 8
-normal platelet count

18.

Hemophilia B

(secondary hemostatic disorder)

-factor 9 def
-same symptoms

19.

Heparin Induced
Thrombocytopenia

-heparin therapy destroys platelets

-fragments can activate other platelets,
leading to thrombosis

20.

HIT Mechanism

-heparin forms complex w/PLatelet Factor

4 (on platelets)
-complex causes development of IgG
autoantibodies

21.

How does ITP

happen

-IgG made in spleen

-bind platelets
-pbound platelets eaten by SPLEEN
macrophages

9.

DIC

-pathologic activation of coag cascade

-microthrombi form

10.

DIC Labs

-low platelet
-high PT and PTT: consuming coag factors
-low fibrinogen: linker molecule used up
-microangiopathic hemolytic anemia
-elevated fibrin split products

11.

DIC
Pathophysio

-increased activation of clotting cascafe

-decreased anticoagulants
-impaired fibrinolysis

12.

DIC Treatment

-fix secondary cause

-blood product transfusion

22.

How to differentiate
Coagulation Factor
Inhibitor and
Hemophilia A

-MIXING STUDY
-Coag F Inhibitor does NOT correct
-Hemophilia A does correct

36.

Tests for 2o Disorders

-PT (extrinsic pathway and

common)
-PTT (intrinsic pathway and
common)

23.

HUS

(primary, quantitative)
-hemolytic uremic syndrome
-e.Coli 0517H7 verotoxin damages
endothelial cells, cause
microthrombi formation
-CHILDREN

37.

tPA

plasminogen to plasmin

38.

Treating Fibrinolysis
Disorders

-Aminocaproic Acid: blocks

activation of plasminogen

39.

Treating Hemophilia A

-give Factor 8

40.

Treating TTP and HUS

PLasmaphersis, corticosteroids

41.

Treating vWF Disease

-desmopressin
-increases vWF release from
Weibel-Palade bodies

42.

Treat ITP

-corticosteroids
-IVIG
-Splenectomy: kills source of IgG
and site of destruction

43.

TTP

(primary, quantitative)
-genetic defect OR autoantibody
formed against ADAMSTS13
-caused by low ADAMS TS13

44.

TTP and HUS Symptoms

-skin, mucosal bleeding

-microangiopathic hemolytic
anemia
-fever
-renal insufficiency
-CNS abnormalitues

45.

Types of Platelet
disorders in 1
hemostasis

Quantitative or qualitative

46.

Uremia

(primary, qualitative)
-poor kidney function, build up of
nitrogenous waste products
-disorder of adhesion and
aggregation

47.

Vitamin K Deficiency

(secondary hemostatic disorder)

-2, 7, 9 10 disrupted
-coagulation not happening

48.

Von Willebrand Disease

(secondary hemostatic disorder)

-vWF def (genetic)
-most common, inherited

49.

Von Willebrand Disease

Labs

-long bleeding time

-high PTT, normal PT
-abnormal Ristocetin test

50.

Von Willebrand Disease

Symptoms

-mucosal and skin bleeding (b/c

platelets cant adhere)

51.

Von Willebrand Disease

Types

quantitative or qualitative
-most common: AD
-platelet adhesion cant occur

52.

What activates VItamin

K

-epoxide reductase
-coumidin blocks^^

24.

ITP Cause

(primary, quantitative)
-IgG attackes platelet antigens (like
gps) -> thrombocytopenia
-common in children and adult

25.

Lab Findings ITP

-low platelet
-normal PT/PTT
-high megakaryocytes

26.

Lab Findings TTP and

HUS

-thrombocytopenia, increased
bleeding time!!
-normal PT/PTT
-anemia + SCHISTOCYTES
-increased megakaryocytes

27.

Large Volume
Transfusions

(secondary hemostatic disorder)

-dilutes coag factors
-results in general deficiency

28.

Mixing Study

-combine normal plasma with

patients plasma
-if CORRECTED: normal plasma
gives back whats deficient, will
reduce PTT (back to normal)
-if NOT CORRECTED: anti-factor Ab
from patient will bind factor in
normal plasma, keeping PTT high

29.

Plasmin

-cleaves fibrin
-cleaves fibrinogen in serum
-destroys coag factors
-blocks platelet aggragtion

30.

PT

-coumidin/warfarin better measured

here

31.

PTT

-measure HEParin effect

32.

Ristocetin test

-give ristocetin to patient platelets,

they will agrregate
-abnormal vWF, no aggregation

33.

34.

35.

Secondary Hemostasis
Disorders

-abnormality in factors
-deep bleeding in muscles or joints
-rebleeding after procedure

Serious Complications
of DIC

microthrombi -> ischemia and

infarcts
bleeding from IV sites and
mucosally because using so much
platelets

Symptoms of 1o
Hemostasis Disorder

-mucosal bleeding
-skin bleeding: easy bruises,
petechiae, ecchymosis (petecheia
is from thrombocytopenia)

53.

What are primary hemostatic disorders characterized by?

Mucosal and Skin Bleeding

-hemoptysis
-**epistaxis**
-menorrhagia
-intracranial bleeding

54.

What causes Vitamin K DEf

-either you have trouble uptaking it, or you lack bacteria that makes it
-newborns, antibiotic therapy, malabsorption, or LIVER FAILURE
-check for liver failure with elevated PT

55.

Whats required to activate factors?

-exposure to activating substance

-phospholipid surface
-Ca+

56.

Where are factors of coag cascade made?

Liver

57.

Why are the factors disrupted in Vitamin K def?

-it gamma carboxylates 2,7, 9 and 10

-promotes coagulation

58.

Why is PTT elevated in VW DIease?

-nee vWF to stabilize factor 8