112 - Coagulation Disorders Flashcards

1
Q

What are the common hemophilia? 2

Remember they are both __ linked

A

Factor 8 deficiency (A)
Factor 9 deficiency (B)
X

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Elongated PT- factor __ deficiency
Elongated aPTT- __ (/) or __ deficiency
Elongated PT and aPTT- factor //_ or __ deficiency

A
7
hemophilia (8/9)
11
2,5,10
fibrinogen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Most hemophilia is type _ (_%). In _% of cases there is no __, and within that group __% are from the mother

A
A
80
30
family history
80
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

In hemophilia the severity of symptoms is correlated with the degree of the ___

A

deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Light deficiency (-% functioning factor)- The bleeding is usually secondary to __, over __% will be identified after it or prior to pre-op blood workout

A

6-30
trauma
25

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Medium deficiency (-% functioning factor) or severe deficiency (

A

1-5
1
hemarthrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

During childhood, patients with hemophilia may experience __ bleeding or when __.

A

cerebral bleeding

circumcised

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

In hemophilia patients, look out for //_ bleedings, as they are life threatening and require immediate treatment.

A

oropharyngeal
CNC
retroperitoneal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The CBC of an hemophilia patient will reveal elongated __ with normal __ time and __. Diagnosis is possible only after specific examination of factors / function

A

aPTT
bleed
platelets
8/9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
How hemophilia patients are treated?
1. 
2. 
3. 
4.
A

recombinant factor
treating acute bleeding
DDAVP
antifibrinolytic drugs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Recombinant factor treatment for hemophilia should keep the factors around _% to avoid __ bleeding- especially to the __

A

1
spontaneous
joints

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Recombinant factor treatment for hemophilia A should be given _ times per week, while in B- _ times per week

A

3

2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

If an acute bleeding has occurred in an hemophilia patient treat with __ ASAP and avoid __treatment.

A

recombinant factors

antiaggregant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

DDAVP is a synthetic __ of __ which increases __ and __ but not __, therefor useful only to __ patients. When used repeatedly may cause __

A
analogue
vasopressin
factor 8
factor 9
hemophilia type A
tachyphylaxis (rapidly diminishing response to successive doses of a drug)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Antifibrinolytic drugs for hemophilia include: 2

A

EACA

tranexamic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Antifibrinolytic drugs for hemophilia are indicated when: 3. There is not indication to use it for __

A

gum bleeding
GI bleeding
oral surgery
hematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Producing __ which __ the factor treatment is a common and significant complication in hemophilia patients. We can identify the problem using __ tests or __ test

A

antibodies
inhibit
plasma mixing
Bethesda

18
Q

Treating bleeding in high responders is done with high concentrations of __ and factors ,,_ or recombinant factor _

A

prothrombin
7,9,10
7

19
Q

Eradication therapy is the most effective treatment for antibodies. It is done with ITI (__)

A

immune tolerance induction

20
Q

Factor 11 is part of the __ pathway and it activates factor __. Patients will bleed when the factor goes under __%

A

intrinsic
9
10

21
Q

Treating factor 11 deficiency is done with __ infusion. In acute bleeding we can use __ drugs. If inhibitors develop- use __/__ or recombinant factor __. Avoid giving __ if inhibitors develop!

A
FFP
antifibrinolytic drugs
PCC/aPCC
7
FFP
22
Q

Dysfibrinogenemia is a rare coagulation disorder, where factor __ is deficient and patients tend to be __

A

7

asymptomatic

23
Q

Coagulation disorders with factor _ or _ deficiency tend to have __ symptoms

A

10

13

24
Q

Rare coagulation disorders tend to present with __ or __ bleeding, rather than __ bleeding

A

membranous
umbilical cord
joint

25
Q

The common causes for DIC: 5

A
bacterial sepsis
malignancy
obstetric 
trauma
drugs
26
Q

The clinical presentation of DIC includes:

A
bleeding
hypercuagobility 
thrombosis
hemodynamic instability (shock)
30-80% mortality (depending on background disease/severity/age)
27
Q

To diagnose DIC we should perform a __ assessment, and repeat after - hours

A

primary

6-8

28
Q

Laboratory examination of a DIC patient will include: 6

A
coagulation (PT, aPTT, thrombin time)- will elongate
lysis markers (FDP, D-Dimer)
reduced function of antithrombin III or plasminogen
29
Q

What are the main DDx for DIC? 3

A

severe liver disease
TTP
immunogenic treatment for malignancies

30
Q

For DIC patients with PT>1.5 of the norm- give __

A

FFP

31
Q

For DIC with low fibrinogen (

A
100
hyperfibrinolysis
cryoprecipitate 
fibrinogen
factor 8
vWF
32
Q

For severe DIC patients give __ transfusion

A

platelets

33
Q

Specific coagulation factors are __ when treating DIC

A

ineffective

34
Q

Light DIC caused by __,__,__ give low dose __

A

solid tumors
APML
thrombosis
heparin

35
Q

When removing stillbirth, or giant hemangioma of a patient with DIC, give- __

A

heparin

36
Q

In severe and acute DIC __ should not be used, as it worsens the bleeding and does not improve survival

A

heparin

37
Q

In acute hyperfibrinolysis in a DIC patient- give ___ drugs (2). Remember to give __ as well to avoid thrombosis. This is especially useful for __ patients

A
antifibrinolytic
EACA
tranexamic acid
heparin
APML
38
Q

DIC patients with an acquired deficiency of __ should be treated with a recombinant version of it. The reasons are: 2

A

protein C
purpura fulminans
meningococcemia

39
Q

What are the main reasons for vitamin K deficiency? 3

A

absorption (surgery/disease)
PBC or other chronic liver disease
newborn

40
Q

Elongated __ is the earliest, most common symptom in vitamin _ deficiency. It is due to lack of __,__,__,__,
Factor 7 has the shortest t1/2 and it elongates the PT before aPTT.

A

PT
K
prothrombin
factor 7/9/10

41
Q

In patients with vitamin K deficiency and active bleeding which requires immediate fixing (pre op)- give __ or __ (which should be avoided in patients with __)

A

PCC
FPP
liver disease

42
Q

In patient with vitamin K deficiency going through life threatening bleeding- give __

A

factor 7a