112 - Coagulation Disorders Flashcards
What are the common hemophilia? 2
Remember they are both __ linked
Factor 8 deficiency (A)
Factor 9 deficiency (B)
X
Elongated PT- factor __ deficiency
Elongated aPTT- __ (/) or __ deficiency
Elongated PT and aPTT- factor //_ or __ deficiency
7 hemophilia (8/9) 11 2,5,10 fibrinogen
Most hemophilia is type _ (_%). In _% of cases there is no __, and within that group __% are from the mother
A 80 30 family history 80
In hemophilia the severity of symptoms is correlated with the degree of the ___
deficiency
Light deficiency (-% functioning factor)- The bleeding is usually secondary to __, over __% will be identified after it or prior to pre-op blood workout
6-30
trauma
25
Medium deficiency (-% functioning factor) or severe deficiency (
1-5
1
hemarthrosis
During childhood, patients with hemophilia may experience __ bleeding or when __.
cerebral bleeding
circumcised
In hemophilia patients, look out for //_ bleedings, as they are life threatening and require immediate treatment.
oropharyngeal
CNC
retroperitoneal
The CBC of an hemophilia patient will reveal elongated __ with normal __ time and __. Diagnosis is possible only after specific examination of factors / function
aPTT
bleed
platelets
8/9
How hemophilia patients are treated? 1. 2. 3. 4.
recombinant factor
treating acute bleeding
DDAVP
antifibrinolytic drugs
Recombinant factor treatment for hemophilia should keep the factors around _% to avoid __ bleeding- especially to the __
1
spontaneous
joints
Recombinant factor treatment for hemophilia A should be given _ times per week, while in B- _ times per week
3
2
If an acute bleeding has occurred in an hemophilia patient treat with __ ASAP and avoid __treatment.
recombinant factors
antiaggregant
DDAVP is a synthetic __ of __ which increases __ and __ but not __, therefor useful only to __ patients. When used repeatedly may cause __
analogue vasopressin factor 8 factor 9 hemophilia type A tachyphylaxis (rapidly diminishing response to successive doses of a drug)
Antifibrinolytic drugs for hemophilia include: 2
EACA
tranexamic acid
Antifibrinolytic drugs for hemophilia are indicated when: 3. There is not indication to use it for __
gum bleeding
GI bleeding
oral surgery
hematuria
Producing __ which __ the factor treatment is a common and significant complication in hemophilia patients. We can identify the problem using __ tests or __ test
antibodies
inhibit
plasma mixing
Bethesda
Treating bleeding in high responders is done with high concentrations of __ and factors ,,_ or recombinant factor _
prothrombin
7,9,10
7
Eradication therapy is the most effective treatment for antibodies. It is done with ITI (__)
immune tolerance induction
Factor 11 is part of the __ pathway and it activates factor __. Patients will bleed when the factor goes under __%
intrinsic
9
10
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!
FFP antifibrinolytic drugs PCC/aPCC 7 FFP
Dysfibrinogenemia is a rare coagulation disorder, where factor __ is deficient and patients tend to be __
7
asymptomatic
Coagulation disorders with factor _ or _ deficiency tend to have __ symptoms
10
13
Rare coagulation disorders tend to present with __ or __ bleeding, rather than __ bleeding
membranous
umbilical cord
joint
The common causes for DIC: 5
bacterial sepsis malignancy obstetric trauma drugs
The clinical presentation of DIC includes:
bleeding hypercuagobility thrombosis hemodynamic instability (shock) 30-80% mortality (depending on background disease/severity/age)
To diagnose DIC we should perform a __ assessment, and repeat after - hours
primary
6-8
Laboratory examination of a DIC patient will include: 6
coagulation (PT, aPTT, thrombin time)- will elongate lysis markers (FDP, D-Dimer) reduced function of antithrombin III or plasminogen
What are the main DDx for DIC? 3
severe liver disease
TTP
immunogenic treatment for malignancies
For DIC patients with PT>1.5 of the norm- give __
FFP
For DIC with low fibrinogen (
100 hyperfibrinolysis cryoprecipitate fibrinogen factor 8 vWF
For severe DIC patients give __ transfusion
platelets
Specific coagulation factors are __ when treating DIC
ineffective
Light DIC caused by __,__,__ give low dose __
solid tumors
APML
thrombosis
heparin
When removing stillbirth, or giant hemangioma of a patient with DIC, give- __
heparin
In severe and acute DIC __ should not be used, as it worsens the bleeding and does not improve survival
heparin
In acute hyperfibrinolysis in a DIC patient- give ___ drugs (2). Remember to give __ as well to avoid thrombosis. This is especially useful for __ patients
antifibrinolytic EACA tranexamic acid heparin APML
DIC patients with an acquired deficiency of __ should be treated with a recombinant version of it. The reasons are: 2
protein C
purpura fulminans
meningococcemia
What are the main reasons for vitamin K deficiency? 3
absorption (surgery/disease)
PBC or other chronic liver disease
newborn
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.
PT
K
prothrombin
factor 7/9/10
In patients with vitamin K deficiency and active bleeding which requires immediate fixing (pre op)- give __ or __ (which should be avoided in patients with __)
PCC
FPP
liver disease
In patient with vitamin K deficiency going through life threatening bleeding- give __
factor 7a
