Professional Documents
Culture Documents
Symptomatic carrier
Spontaneous mutation in a carrier
Mild >5%
Moderate 1-5%
Severe <1%
3.
Hemophilia
4.
What disease is
associated with a normal
exam except for bruises
and Petechiae and a
normal CBC except for
low platelets?
5.
What disease is
associated with
consumption of factors
including factors VII, IX,
and VIII?
DIC
6.
7.
8.
Factor V deficiency
9.
X linked
10.
What is Glanzmann
thrombasthenia
associated with?
11.
Vit K
12.
13.
Steroids
2.
14.
~20%
15.
Type 2
16.
Type 1
17.
Type 3
18.
Factor XII
19.
Factor VII
20.
Bernard
Soulier
Syndrome
Acquired
inhibition of XIII
2.
Antophosphoipid
Antibody
Syndromes
(APLS)
3.
Autoimmune
Inhibitors of F
VIII
4.
Circulating
Anticoag: Types
5.
Circulation
Anticoagulants
6.
Factor X
deficiencies
7.
FIX Inhibitors
8.
11.
FVIII:C
inhibitors
Autoantibodies
12.
Heparin binds
autoprothrombin
III
13.
Heparin therapy
14.
Inhibitors VIII:C
15.
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
17.
Nonspecific
inhibitors
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
10.
FVIII:C
inhibitors
Alloantibodies
18.
Specific Inhibitors
19.
20.
VIII:C Treatment
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
23.
PT prolonged
APTT possibly prolonged
TT N
Fibrinogen N
Acute ITP
-children
-self limited
-after immunization or virus
2.
ADAMS TS13
3.
Alpha 2
Antiplasmin
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
15.
Fibrinolysis
Disorders
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
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
18.
Hemophilia B
19.
Heparin Induced
Thrombocytopenia
20.
HIT Mechanism
21.
9.
DIC
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
12.
DIC Treatment
22.
How to differentiate
Coagulation Factor
Inhibitor and
Hemophilia A
-MIXING STUDY
-Coag F Inhibitor does NOT correct
-Hemophilia A does correct
36.
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
39.
Treating Hemophilia A
-give Factor 8
40.
PLasmaphersis, corticosteroids
41.
-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.
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
48.
49.
50.
51.
quantitative or qualitative
-most common: AD
-platelet adhesion cant occur
52.
-epoxide reductase
-coumidin blocks^^
24.
ITP Cause
(primary, quantitative)
-IgG attackes platelet antigens (like
gps) -> thrombocytopenia
-common in children and adult
25.
-low platelet
-normal PT/PTT
-high megakaryocytes
26.
-thrombocytopenia, increased
bleeding time!!
-normal PT/PTT
-anemia + SCHISTOCYTES
-increased megakaryocytes
27.
Large Volume
Transfusions
28.
Mixing Study
29.
Plasmin
-cleaves fibrin
-cleaves fibrinogen in serum
-destroys coag factors
-blocks platelet aggragtion
30.
PT
31.
PTT
32.
Ristocetin test
33.
34.
35.
Secondary Hemostasis
Disorders
-abnormality in factors
-deep bleeding in muscles or joints
-rebleeding after procedure
Serious Complications
of DIC
Symptoms of 1o
Hemostasis Disorder
-mucosal bleeding
-skin bleeding: easy bruises,
petechiae, ecchymosis (petecheia
is from thrombocytopenia)
53.
54.
-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.
56.
Liver
57.
58.
...
2.
acquired
hemophilia
3.
antibodies
4.
FEIBA
5.
secondary
hemostasis
6.
Individual factor
levels
Von Willebrand
panel
7.
D-dimers
Thrombin time
Inhibitor titers
8.
prolonged
9.
Schistocytes
10.
liver disease
11.
Muscle
hemorrhage
Deep soft tissue
hematomas
CNS bleedingwith trauma
12.
Secondary
hemostatic
13.
uncommon!!
14.
Acquired clotting
defects
15.
prenatal
16.
hemostatic
17.
Splenomegaly;
sequestration of
platelets
18.
acute bleeding
19.
Clinical
manifestations of
____:
Easy bruising
Bleeding form
mucous
membranes
(gum bleeding
nosebleeds
(epistaxis)
Menorrhagia)
Bleeding after
surgery or trauma
20.
Coagulation
defects due to
liver disease are
only treated if:
21.
Coagulation
defects in liver
disease
...
22.
23.
Cryoprecipitate
includes:
Fibrinogen
Factor VIII
vWF
Factor XIII
24.
DDAVP MOA:
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)
36.
acquired
bleeding/hemophilia
37.
38.
inheritance pattern
39.
FEIBA= ____
factor eight
inhibitor bypass
activity
40.
acquired inhibitors
of coagulation
41.
Desmopressin
(DDAVP)
42.
Epsilon-amino
caproic acid;
Tranexamic acid
43.
Plasma-derived
products Humate-P
Alphanate
Wilate
44.
OCPs (oral
contraceptive pills)
45.
PFA-100; PTT
46.
clinical
presentation;
intrinsic
47.
Factor VIII
48.
Hemophilia B AKA
Christmas disease
49.
Factor IX
50.
spontaneous
mutation
51.
X-linked recessive
52.
males
53.
vitamin K
54.
less
DIC leads to
excessive ____
production which
causes fibrin clots
to be deposited in
vessels and block
vital circulation
thrombin
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
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.
RBCs
66.
platelets
67.
In DIC:
PT/PTT is ____
Fibrinogen is ____
D-dimers is ____
Thrombin time is ____
Increased
Decreased
Increased
Increased
68.
Prophylactic
factor
69.
vitamin K
70.
larger (knees,
hips, elbows,
shoulders,
ankles..)
71.
large and
intermediate
multimers
72.
large multimers
73.
NORMAL
74.
NORMAL
75.
Type 2N
76.
Type 2B
77.
Type 2M
78.
Disemminated
intravascular
coagulation (DIC)
Important to
consider history of
factors
predisposing to
liver disease such
as ____, ____, and
iron overload
61.
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.
post-trauma;
surgery
90.
spontaneous;
trauma
91.
92.
Type I
93.
autosomal
recessive
94.
Secondary
hemostatic
defect
95.
placental
abruption
placenta
previa
96.
DIC
97.
hemarthrosisjoint bleeding
98.
cryoprecipitate
99.
secondary
100.
primary
101.
von Willebrand
102.
normal
103.
bleeding time
104.
monoclonal
antibodies
105.
Humate-P;
Alphanate;
Wilate
106.
primary
hemostasis
107.
normal
108.
veins
109.
normal
110.
PT; PTT
111.
very high
History
____ is the
degradation of
cartilage joint in
hemophilia
patients; it
becomes
destroyed and not
functional
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:
Medication
history, such as
____, and food
intake, especially
vitamin ____, are
important for
studying bleeding
patients
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=
116.
Screening studies
for bleeding
patients (3):
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)
121.
Spontaneous
hemarthrosis
with severe
hemophilia
(image)
122.
Subendothelial
vWF interacts
with the ____
platelet receptor
GpIb
123.
Supernatant of
FFP is used to
make ____
124.
Supernatant of
thawed FFP
contains four
factors:
125.
Tests specific to
VWD (all
available, not all
necessarily
used):
VWF activity
VWF antigen
Factor VIII level
RIPA
VWF multimers and pattern
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.
absence
131.
autosomal recessive
very uncommon, very
severe
132.
autosomal dominant
133.
qualitative
134.
quantitative
135.
autosomal dominant
136.
joint bleeding
137.
mucosal
138.
8-12 hours
*now have better ones
139.
24 hours
140.
PT; PTT
141.
142.
warfarin
143.
rapidly
144.
gamma-carboxylation
145.
nerve
146.
congenital
147.
Type 2A
148.
normal
149.
150.
megakaryocytes
151.
Coagulopathy and
thrombocytopenia are
usually mild