Inherited vs Acquired Coagulation Disorders

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1.

How do women show

signs of hemophilia?

Symptomatic carrier
Spontaneous mutation in a carrier

What are the percent

activity of factor 8 or 9
associated with severity
of hemophilia?

Mild >5%
Moderate 1-5%
Severe <1%

3.

What bleeding disorder is

associated with joint
problems?

Hemophilia

4.

What disease is
associated with a normal
exam except for bruises
and Petechiae and a
normal CBC except for
low platelets?

Immune thrombocytopenia purpura

5.

What disease is
associated with
consumption of factors
including factors VII, IX,
and VIII?

DIC

6.

What do patients with

Glanzmann
thrombasthenia respond
to, platelet agonist or
ristocetin?

Lack of response to platelet

agonist, response to ristocetin

7.

What factor deficiency is

associated with
amyloidosis in adult
patients?

Factor X deficiency due to

absorption of Factor X

8.

What factor deficiency

may be combined with
Factor 8 deficiency due
to an abnormal
chaperone protein from
ER to Golgi apparatus?

Factor V deficiency

9.

What inheritance type is

associated with
hemophilia?

X linked

10.

What is Glanzmann
thrombasthenia
associated with?

Platelet Deficiency in IIb3

integrin - fibrinogen receptor that
leads to no fibrinogen bridging of
platelets to other platelets can
occur, and the bleeding time is
significantly prolonged

11.

What is necessary for

gamma-carboxylation of
factors II,VII, IX, and X?

Vit K

12.

What is the stabilizer for

Factor 8?

VWF. Deficiency in VWF leads to

deficiency in Factor 8

13.

What is the standard

therapy for Immune
thrombocytopenia
purpura?

Steroids

2.

14.

What percent of hemophilia occurs from

spontaneous mutations?

~20%

15.

What type of VWD has an absence of high

multimer weight of VWD protein?

Type 2

16.

What VWD type is associated with any degree of

reduced levels of VWF (most common and
usually the mildest form)?

Type 1

17.

What VWD type is associated with virtual

absence of VWF (the most severe form, only
occurring in 1 to 3 persons per million)?

Type 3

18.

Which factor deficiency is NOT associated with

bleeding?

Factor XII

19.

Which factor has the shortest half life?

Factor VII

20.

Which syndrome is opposite of Glanzmann

thrombasthenia and can't bind ristocetin?

Bernard
Soulier
Syndrome

Acquired Coagulation Disorders

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1.

Acquired
inhibition of XIII

following isoniazid drug therapy for

tuberculosis

2.

Antophosphoipid
Antibody
Syndromes
(APLS)

acquired coag defect associated w

thrombosis
aPL - antophopholipid Ab
aCL - anticardiolipin Ab (false pos w VDRL
test)
LAC - LA
CAPS - catastrophic antiphospholipid
syndrome

3.

Autoimmune
Inhibitors of F
VIII

Acquired VIII:R (vWD)

may be seen with autoimmune disease or
lymphoproliferative disorder

4.

Circulating
Anticoag: Types

Specific inhibitors - specific for particular

factors
Nonspecific - interfere w phospholipid
components of reagents

5.

Circulation
Anticoagulants

develop with underlying disorders or spont

composed of imunoglobulins
allo or auto antibodies

6.

Factor X
deficiencies

occurs rarely in people with amyloidosis

7.

FIX Inhibitors

rare, due to transfusion, classified as

alloAb
VIII:C and IX, Ab do not increase bleeding
frequency, rather hemarthosis, muscle, and
soft tissue hemorrhages are symtpoms
Suspected in hemophiliac if transfused
factor replacement products appear to have
reduced effectiveness, hemostasis is hard
to achieve, or both

8.

FIX Lab Test

11.

FVIII:C
inhibitors
Autoantibodies

most often seen in patients w lupus, RA,

drug reactions, mm, ex
aka aquire hemophilia
Symptoms: lg hematomas, gross hematuria,
bleeding around pharyngeal or peritoneal
cavities, cerebral hemorrhages,
hemarthrosis
Should be suspected in anyone w no prior
history who present w massive bruising or
hematoma
mortality rate 20%

12.

Heparin binds
autoprothrombin
III

greatly enhances ability to bind and

inactivate thrombin

13.

Heparin therapy

fost, potent anticoag

commonly used to treat thrombosis
APTT used to monitor heparin therapy
TT also prolonged bc affected by heparin
and FDPs
Occasionally PT is prolonged if patient has
recived heparin for a long period
PT, APTT, TT greatly prolonged
*confirmed by protamin sulfate (binds to
heparin and removes it)

14.

Inhibitors VIII:C

most result from factor concentrate

transfusions
autoantibodies
IgG antibodies
Do not interfere with function of vWF or BT
Inhibitors in hemophiliacs when VIII has
short half life

15.

LAC Lab Test

PT and TT N
APTT prolonged
mixing study no corection w normal plasma
Extended incubation extends APTT
proportionally to incubation time
Factor Assay N

16.

LAC - Lupus-like
anticoagulant

aka Antiphospholipid Antibody Syndrome

autoantibodies react against phospholipid
portion of APTT reagent
Develops in 31% of patients w SLE, taking
hemothiazine, lymphoproliferative disorders
more frequently associated w venous
thrombosis than arterial

17.

Nonspecific
inhibitors

usually accidentally discovered w prolonged

APTT screening test
Mixing studies = no correction w normal
pool plasma
IgG or IgM interfere w phopholipid
dependent tests

PT N
APTT prolonged
** Mixing study no correction w normal pool
plasma
** Time and Temp Dependency
2Hr incubation at 37 shows prolongation

9.

FIX Treatment

First stabilize hemostasis

Goal - rid body of Ab
low titer inhibitor
- give high conc of VIII:C to overwhelm Ab
and all of binding sites = Bethesda titer
- porcine recombinant FVIII:C concentrates
High titer
- steroids, porcine concentrates,
immunosuppressive, cytotoxic agents,
plasmapheresis

10.

FVIII:C
inhibitors
Alloantibodies

IgG to FVIII/vEF complex

Does not interfere w function of vWF so BT
is normal
frequently encountered
due to transfusion

in vitro - prolongation of tests

in vivo - hypercoagulable state

18.

Specific Inhibitors

Antibody specific to factor

ABs directly inhibit factor activity or cause inc clearance
Secondary to replacement therapy/transfusion or arise spont wo disorder

19.

Time and Temp Dependency

Initial test: plt + normal pooled plasma

Normal person = correction
Inhibitor = prolonged / no correction
Incubation control: 37 degrees 2 hours
#1 patient
#2 normal pooled plasma
no prolongation indicates inhibitors effect on FVIII:C provided in normal pool plasma

20.

VIII:C Treatment

VIII concentrates (have to give enough to overcome inhibitor

steroids
imunosuppressive therapy
cytotoxic agents
prothrombin-complex concntrates
plasmapheresis in severe cases

21.

Vit K causes

poor diet
biliary obstruction, not absorbing
intestinal malabsorption diseases
gut sterilization
hemorrhagic disease of the newborn
coumadin therapy

22.

Vit K deficiency

Results in impaired synthesis of II, VII, IX, X. Protein C and S

23.

Vit K Lab Test

PT prolonged
APTT possibly prolonged
TT N
Fibrinogen N

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

22- Congenital and Acquired Coagulation Disorders

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1.

Acquired deficiency in clotting factors due

to decreased synthesis:

...

2.

Acquired inhibition of coagulation is

sometimes called "____"

acquired
hemophilia

3.

Acquired inhibitors of coagulation are

____ to coagulation factors, usually factor
8

antibodies

4.

Activated factors 2, 7, 9, and 10 are also

called ____

FEIBA

5.

Activation of coagulation system=

secondary
hemostasis

6.

After screening, go to definitive studies

including (2):

Individual factor
levels
Von Willebrand
panel

7.

Ancillary studies can be done to define

more (after general and specific tests) (3):

D-dimers
Thrombin time
Inhibitor titers

8.

aPTT in hemophilia is usually ____

prolonged

9.

____ are the type of RBCs seen in

peripheral blood smear of DIC pt

Schistocytes

10.

Arterial nevi, splenomegaly, palmar

erythema are stigmata of ____

liver disease

11.

Aside from hemarthrosis, 3 S&S of

hemophilia:

Muscle
hemorrhage
Deep soft tissue
hematomas
CNS bleedingwith trauma

Note: secondary hemostasis defects

12.

____ bleeding pattern includes deep

hematomas, muscle bleeding, bleeding
with surgey/trauma

Secondary
hemostatic

13.

Bruising and mucosal bleeding such as

epistaxis and gum bleeding is ____ in
hemophilia

uncommon!!

14.

____ can also be due to excessive

destruction of coagulation factors

Acquired clotting
defects

15.

Carrier testing and ____ diagnosis are

available for hemophilia using RFLP

prenatal

16.

Cirrhosis of liver => splenomegaly =>

combined primary and secondary ____
defects

hemostatic

17.

Cirrhosis of the liver can have what effect

on the spleen and why?

Splenomegaly;
sequestration of
platelets

18.

Clinical circumstance predisposing to DIC

are seen in ____

acute bleeding

19.

Clinical
manifestations of
____:
Easy bruising
Bleeding form
mucous
membranes
(gum bleeding
nosebleeds
(epistaxis)
Menorrhagia)
Bleeding after
surgery or trauma

von Willebrand Disease (VWD)

20.

Coagulation
defects due to
liver disease are
only treated if:

bleeding OR procedure is planned

21.

Coagulation
defects in liver
disease

...

22.

____ contains ALL

factors (a type of
treatment)

FFP (fresh frozen plasma)

23.

Cryoprecipitate
includes:

Fibrinogen
Factor VIII
vWF
Factor XIII

24.

DDAVP MOA:

releases stored vWF from endothelial

cells and platelets

25.

Deep muscle
bleeds can lead to
____ => Volkman's
ischemic
contracture (nerve
damage if
compartment
syndrome is not
corrected)

compartment syndrome
26.

Defective
synthetic function
in liver causes
____

coagulopathy

27.

28.

29.

30.

Degrees of
severity of
hemophilia:
1) Severe: <____%
factor level
2) Moderate ________% factor level
3) Mild ________% factor level

1; 1-5; 5-30

Desmopressin
acetate (DDAVP) is
NOT effective in
____

hemophilia B

DIC especially
uses up which
clotting factor?

Fibrinogen (1)

DIC has what

impact on clotting
factors and
platelets?

Uses up clotting factors and platelets

(Global consumption of coagulation
factors, esp. fibrinogen)

36.

Factors contributing to liver disease

could be indicative of ____

acquired
bleeding/hemophilia

37.

Factor VIII and Factor IX in hemophilia

is ____

ONE or the other is

reduced!

38.

Family history is important to

determine the ____

inheritance pattern

39.

FEIBA= ____

factor eight
inhibitor bypass
activity

40.

FEIBA is used to treat ____

acquired inhibitors
of coagulation

41.

For mild hemophilia A, always consider

administering ____ inhaled or IV

Desmopressin
(DDAVP)

42.

For prevention of fibrinolysis and

mucous membrane bleeding only,
treatment include ____ and ____

Epsilon-amino
caproic acid;
Tranexamic acid

43.

For surgery/severe loss in VWD,

replacement of vWF with ____ is a
possible treatment

Plasma-derived
products Humate-P
Alphanate
Wilate

44.

General measures for treating VWD

include ____ and topical agents

OCPs (oral
contraceptive pills)

45.

General screening tests used in

diagnosing VWD are ____ and ____

PFA-100; PTT

46.

Hemophilia A and B are identical in

____ and are both defects in the ____
pathway

clinical
presentation;
intrinsic

47.

Hemophilia A- MOST common, is a

defect in ____

Factor VIII

48.

Hemophilia B AKA

Christmas disease

49.

Hemophilia B is a defect in ____

Factor IX

50.

Hemophilia genes have a high rate of

____, leading to 1/3 new cases with a
negative family history

spontaneous
mutation

51.

Hemophilia is a ____ inheritance

disease, but about 30% cases are new
mutations

X-linked recessive

52.

Hemophilia is almost exclusively found

in (males/females)

males

53.

Hospitalized patients with poor oral

intake, often develop ____ deficiency,
especially if on antibiotics! This
impacts production of clotting factors

vitamin K

54.

However, an advantage to using on

demand factor is ____ factor is used

less

DIC leads to
excessive ____
production which
causes fibrin clots
to be deposited in
vessels and block
vital circulation

thrombin

The ____ DIC

usually only
requires treatment
of underlying
condition- retained
dead fetus,
adenocarcinoma

rare chronic

Disadvantage to
using "on demand"
factor treatment is
it is unpredictable
and more likely to
cause ____ over
time

joint damage

34.

Dissolution of the
clot- fibrinolysis=

Tertiary hemostasis

55.

35.

Extensive tissue
damages include:
Get familiar with

acute hemolytic transfusion reaction

tumor lysis syndrome
fat embolism
heat stroke

How to treat DIC?

1) Urgently address ____
2) Replacement of ____
3) Platelet transfusion for bleeding

Underlying cause;
Depleted
coagulation
factors, especially
fibrinogen

56.

Iatrogenic=

caused by doctor

31.

32.

33.

57.

58.

Iatrogenic
complications in
hemophilia can
occur in
circumcision, i.m.
injection, i.j.
stick. Image:

If a forearm
compartment
syndrome is not
corrected, it can
lead to ____

65.

In DIC, fibrin stands can sheer apart ____

causing microangiopathic hemolytic
anemia

RBCs

66.

In DIC patient's peripheral blood smear,

you may also see an absence of ____
because they have been used up

platelets

67.

In DIC:
PT/PTT is ____
Fibrinogen is ____
D-dimers is ____
Thrombin time is ____

Increased
Decreased
Increased
Increased

68.

____ in hemophilia is standard of care

Prophylactic
factor

69.

In neonates, routinely give IM injection of

____ at delivery to prevent acquired
clotting defciency

vitamin K

70.

In progressive hemophilic arthropathy,

usually ____ joints are affected

larger (knees,
hips, elbows,
shoulders,
ankles..)

71.

In Type 2A VWD, there is an absence of

____

large and
intermediate
multimers

72.

In Type 2B VWD, there is an absence of

____

large multimers

73.

In Type 2M, you see a ____ multimer

pattern

NORMAL

74.

In Type 2N VWD, there are ____ multimer

NORMAL

75.

In ____ VWD, there is a decreased affinity

for factor VIII

Type 2N

76.

In ____ VWD, there is an increased

affinity for GpIb (gain of function
mutation)

Type 2B

77.

In ____ VWD, there is decreased plateletdependent function

Type 2M

78.

____ is a medical emergency where

coagulation initiated within the vascular
system

Disemminated
intravascular
coagulation (DIC)

Volkman's ischemic contracture

If it a less
common type of
VWD and the first
three tests do not
clarify diagnosis,
can move on to the
other two tests:
____ and ____

RIPA; VWF multimers + pattern

Important to
consider history of
factors
predisposing to
liver disease such
as ____, ____, and
iron overload

alcohol; chronic hepatitis

61.

In 2014, now have

modified factors
with longer ____

half-life

62.

In acute situation,
the pattern and
clinical scenario
of DIC should be
questioned

...

63.

In addition to
giving factors, you
want to also treat
acquired inhibitors
of coagulation by
____

immunosuppression (reduces Ab
production)

64.

In diagnosing
VWD, only ____,
____, and ____
tests are needed if
the pattern is
autosomal
dominant AND
there is a
proportional
reduction in all
three levels

VWF antigen
VWF activity
Factor VIII level

59.

60.

79.

____ is most
important aspect
of understanding
the patient's
bleeding problem

89.

Mild hemophilia pts have bleeding ____ or

____ only

post-trauma;
surgery

90.

Moderate hemophilia pts have spontaneous

bleeding, or bleeding post-____

spontaneous;
trauma

91.

More commonly factor ____ will be

reduced in hemophilia

92.

Most common type of VWD?

Type I

93.

Most uncommon clotting factor deficiencies

have a ____ inheritance pattern and are
RARE

autosomal
recessive

94.

Most uncommon coagulation factor

deficiencies present with ____ bleeding
pattern

Secondary
hemostatic
defect

95.

Obstetric "catastrophes" include ____ and

____

placental
abruption
placenta
previa

96.

Obstetric catastrophes, trauma, crush

injuries, closed head injuries,
malignancies, and extensive tissue damage
can all cause ____

DIC

97.

One key clinical sign of hemophilia is ____

hemarthrosisjoint bleeding

98.

Other part of the FFP is the ____

cryoprecipitate

99.

Pattern of muscle/deep bleeding suggests

defect in ____ hemostasis- coagulopathy

secondary

100.

Pattern of skin and mucosal bleeding seen

in defect of ____ hemostasis

primary

101.

Patterns seen in defects of primary

hemostasis suggest ____ disease,
thrombocytopenia, and platelet functional
defect

von Willebrand

102.

PFA-100 in hemophilia is ____

normal

103.

PFA-100 replaces ____ (outdated test)

bleeding time

104.

Plasma-derived factors for hemophilia are

purified with ____

monoclonal
antibodies

105.

Plasma-derived products used to treat VWD

include ____, ____, and ____

Humate-P;
Alphanate;
Wilate

106.

Platelet adhesion and aggregation=

primary
hemostasis

107.

Platelet count in hemophilia is ____

normal

108.

Primary or secondary prophylaxis

administeration requires good ____

veins

109.

PT in hemophilia is usually ____

normal

110.

(PT/PTT) will be more marked than

(PT/PTT), since the half-life of factor VII is
shorter

PT; PTT

111.

The PTT in acquired inhibitors of

coagulation is ____

very high

History
____ is the
degradation of
cartilage joint in
hemophilia
patients; it
becomes
destroyed and not
functional

Progressive hemophilic arthropathy

81.

____ is used to
treat VWD Type I

Desmopressin

82.

It takes ____
factor use to treat
hemophilia
prophylactically,
but it does make
for a better
lifestyle

high

Liver synthesizes
all coagulation
factors except
____

factor 8

84.

Low albumin is a
sign of altered
____ function

liver

85.

The lower/later
position of factor
that is deficient is
usually more
____, for example
fibrinogen

severe

86.

MAIN cause of
DIC:

sepsis

87.

Main treatment
goal in acquired
inhibitors of
coagulation is to:

bypass the coagulation factor to which

antibody is directed to (usually factor
VIII)

Medication
history, such as
____, and food
intake, especially
vitamin ____, are
important for
studying bleeding
patients

warfarin (anticoagulant); vitamin K

80.

83.

88.

112.

Recombinant
factor ____
initiates
coagulation via
the extrinsic
pathway and is
used to treat
acquired
inhibitors of
coagulation

VIIa

113.

Recombinant
factors are
available for
hemophilia but
are more ____

expensive

114.

Recombinant
factors available
for what 3
factors?

VIIa
VIII
IX

115.

RIPA=

Ristocetin-induced platelet aggregation

116.

Screening studies
for bleeding
patients (3):

PT, PTT, CBC

Severe
hemophilia pts
have ____
bleeding

spontaneous

Since vitamin K
deficiency affects
all pathways of
coagulation
cascade, both PT
and PTT will be
____

Elevated

Some patients
prefer "on
demand" factor
treatment, where
they wait for ____
to occur before
giving
themselves factor

bleeding

117.

118.

119.

120.

Spontaneous head
bump with severe
hemophilia
(image)

*now totally blood-free

121.

Spontaneous
hemarthrosis
with severe
hemophilia
(image)

122.

Subendothelial
vWF interacts
with the ____
platelet receptor

GpIb
123.

Supernatant of
FFP is used to
make ____

PCC (prothrombin complex concentrate)

124.

Supernatant of
thawed FFP
contains four
factors:

Factor 2, 7, 9, 10 (vit-K dependent)

125.

Tests specific to
VWD (all
available, not all
necessarily
used):

VWF activity
VWF antigen
Factor VIII level
RIPA
VWF multimers and pattern

Get familiar with

list
126.

Three tools for

evaluating patient
you suspect
having a
coagulation
disorder:

1) Medical history
2) Physical exam
3) Lab studies

127.

To replace coag.
factors, esp.
fibrinogen, in DIC
patients,
treatment is
usually admin of
____

cryoprecipitate

128.

Treatment for
hemophilia:
____ factors or
recombinant
factors

plasma-derived

129.

Treatment of
VWD- only on
demand

Desmopressin (DDAVP)
Contraindication: Type 2B!!

130.

Type III is an ____ of vWF

absence

131.

Type III is ____ inheritance pattern

autosomal recessive
very uncommon, very
severe

132.

Type II is ____ inheritance pattern

autosomal dominant

133.

Type II VWD is a ____ deficiency of

vWF

qualitative

134.

Type I VWD is a ____ deficiency of

vWF

quantitative

135.

Type I VWD is an ____ inheritance

pattern

autosomal dominant

136.

Uncommon coagulation factor

deficiencies do not show ____ in
the common clinical
symptoms/signs

joint bleeding

137.

Uncommon S&S of hemophilia:

epistaxis, other ____ bleeding,
prolonged bleeding from cuts,
petechiae

mucosal

138.

Usually factor 8 circulates (halflife) for ____

8-12 hours
*now have better ones

139.

Usually half-life of factor 9 is ____

24 hours

140.

Usually (PT/PTT) >> (PT/PTT) with

coagulation defects in liver disease

PT; PTT

141.

Variable clinical presention

depends on ____ factor level, and
relates to position of ____

baseline; factor in the

pathway

142.

Vitamin K deficiency can occur in a

patient on vitamin K antagonists
such as ____

warfarin

143.

Vitamin K deficiency treated with

vitamin K administration responds
____

rapidly

144.

Vitamin K important for ____ of

factor 2, 7, 9, 10

gamma-carboxylation

145.

Volkman's ischemic contracture is

____ damage when compartment
syndrome not corrected

nerve

146.

Von Willebrand Disease is a ____

coagulation factor deficiency

congenital

147.

____ VWD is impaired secretion or

increased proteolysis

Type 2A

148.

vWF in hemophilia is ____

normal

149.

VWF is very important for two

functions:

1) Carrier for factor

VIII (prevents it from
proteolysis= longer
half-life)
2) Cross-links platelets
to exposed collagen
AND to each other

150.

VWF made in ____

megakaryocytes

151.

Why is rare chronic DIC only

treated with addressing underlying
condition?

Coagulopathy and
thrombocytopenia are
usually mild