112 - Coagulation Disorders

Question 1

What are the common hemophilia? 2

Remember they are both __ linked

Answer 1

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

Question 2

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

Answer 2

7
hemophilia (8/9)
11
2,5,10
fibrinogen

Question 3

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

Answer 3

A
80
30
family history
80

Question 4

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

Answer 4

deficiency

Question 5

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

Answer 5

6-30
trauma
25

Question 6

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

Answer 6

1-5
1
hemarthrosis

Question 7

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

Answer 7

cerebral bleeding

circumcised

Question 8

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

Answer 8

oropharyngeal
CNC
retroperitoneal

Question 9

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

Answer 9

aPTT
bleed
platelets
8/9

Question 10

How hemophilia patients are treated?
1. 
2. 
3. 
4.

Answer 10

recombinant factor
treating acute bleeding
DDAVP
antifibrinolytic drugs

Question 11

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

Answer 11

1
spontaneous
joints

Question 12

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

Answer 12

3

2

Question 13

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

Answer 13

recombinant factors

antiaggregant

Question 14

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

Answer 14

analogue
vasopressin
factor 8
factor 9
hemophilia type A
tachyphylaxis (rapidly diminishing response to successive doses of a drug)

Question 15

Antifibrinolytic drugs for hemophilia include: 2

Answer 15

EACA

tranexamic acid

Question 16

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

Answer 16

gum bleeding
GI bleeding
oral surgery
hematuria

Question 17

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

Answer 17

antibodies
inhibit
plasma mixing
Bethesda

Question 18

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

Answer 18

prothrombin
7,9,10
7

Question 19

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

Answer 19

immune tolerance induction

Question 20

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

Answer 20

intrinsic
9
10

Question 21

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!

Answer 21

FFP
antifibrinolytic drugs
PCC/aPCC
7
FFP

Question 22

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

Answer 22

7

asymptomatic

Question 23

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

Answer 23

10

13

Question 24

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

Answer 24

membranous
umbilical cord
joint

Question 25

The common causes for DIC: 5

Answer 25

``` bacterial sepsis malignancy obstetric trauma drugs ```

Question 26

The clinical presentation of DIC includes:

Answer 26

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

Question 27

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

Answer 27

primary | 6-8

Question 28

Laboratory examination of a DIC patient will include: 6

Answer 28

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

Question 29

What are the main DDx for DIC? 3

Answer 29

severe liver disease TTP immunogenic treatment for malignancies

Question 30

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

Answer 30

FFP

Question 31

For DIC with low fibrinogen (

Answer 31

``` 100 hyperfibrinolysis cryoprecipitate fibrinogen factor 8 vWF ```

Question 32

For severe DIC patients give __ transfusion

Answer 32

platelets

Question 33

Specific coagulation factors are __ when treating DIC

Answer 33

ineffective

Question 34

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

Answer 34

solid tumors APML thrombosis heparin

Question 35

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

Answer 35

heparin

Question 36

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

Answer 36

heparin

Question 37

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

Answer 37

``` antifibrinolytic EACA tranexamic acid heparin APML ```

Question 38

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

Answer 38

protein C purpura fulminans meningococcemia

Question 39

What are the main reasons for vitamin K deficiency? 3

Answer 39

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

Question 40

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.

Answer 40

PT K prothrombin factor 7/9/10

Question 41

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

Answer 41

PCC FPP liver disease

Question 42

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

Answer 42

factor 7a