Bleeding Disorders Flashcards
types of Hemophilia
what is christmas disease?
what does christmas remind u of? RED COLOURS
Haemophilia B
This is a deficiency in factor 9
How is Christmas disease inherited?
it is X-linked recessive,
this means that MEN will have it for sure bc they have only one X
while women have 2 x’s, so the othere can be healthy! CARRIERS
(christmas= Hemphilia B)
Hemophilila A
Explain the mode of inheritance, the basic clinical and laboratory abnormalities
of patients.
- x-linked
- congenital lack of Factor 8
-Intrinsic pathway defect!
Symptoms
Hemarthroses (bleeding into joints ex:Knee),
bruising easily ,
bleeding after trauma and surgery and teeth surgery
Findings
PTT ^
PT normal
Treatments
give desmopressin DDAVP>> stimulates VWF release, to stabilize factor 8
or
inject missing clotting factors>
(but in those with severe hemohpilila like complete ansence of that clotting factor!, the body can recognise it as foreign and make antibodies against them!)
symptoms of hemophilila
Explain the tests in a coagulation screening
just an overview of hemophilila A & B
ok
Von Willebrand’s Disease
what is the function of VWf?
Explain the mode of inheritance, the basic clinical, treatments and laboratory abnormalities
of patients.
VWF carries Factor 8 and mediates platelet adhesion to endothelium
- Intrinsic pathway defect
- autosomal dominant
- males and females affected
- genetics defects cause it
- defect in platelet plug formation
Symptoms
skin and mucous membrane bleeding
- bleeding gums! bruising!
- prolonged bleeding after trauma!
- heavy periods
- post surgey
- post dental extraction
Findings
PT normal
PPT ^
Treatment
Desmopressin> relaxes vWF stored in endothelium (boosts amount of factors)
BUT SPONTANEOUS JOINT OR MUSCLE BLEEDS R RARE!
Describe the causes, presentation, consequences and treatment of
disseminated intravascular coagulation DIC
when hemostasis starts to run out of control! (pathalogical activation of coagulation)
lots of blood clots start to form! (microthrombi) laying down fibrin
blood cells passing through these clotted vessels r damaged (hemolysis)> shistocytes
this consumes all the clotting factors and platelets!
U GET BLEEDING!
Paradoxily, Patients passes through 2 stages, 1st coagulant then Anticoagulant
It NEVER occurs as a disease in itself but is always a complication of another condition!
Causes
STOP Making New Thrombin
Sepsis (gram-)
Trauma
Obstetric causes – placental abruption, pre- eclampsia, amniotic fluid embolism
Pancreatits acute
Malignancy
Nephrotic syndrome
Transfusion
Lab findings:
shistocytes (due to damages RBC cause by passing through the clottled areas)
^ fibrin degredation products (d-dimers)
low fibrinogen
Low factor 5 & 8 (co factors)
Treatments:
focus on underlying cause
support various organs!
- ventilation
- hemodynamic
- transfusions
Describe the Lab findings of DIC
describe types of thrombocytopenia
SPUD
A patient presents with theses symptoms
what is the most likely diagnosis for these findings?
Describe it
how is it treated?
Which coagulation tests will be abnormal and which normal? Why?
Immune thrombocytopenic purpura (ITP)
your body makes Anti-Gp2b/3a antibodies to attack the platelets (recepter on platelet) .
LOW PLATLETS
Treatment
corticosteroids
IVIG
do not inject platelets cuz theyll be destroyed by the body anyways -,-
Bleeding time is prolonged, but PT and PTT is usually normal, thats mainly because bleeding time is an assessment of platelet function, while PT and PTT is a clotting factor issue, and this guy doesn’t have a clotting factor problem.
What is Trousseau’s syndrome? Why does it occur?
,
Describe the causes and consequences of thrombocytopenia
Patients generally not symptomatic until the platelet count is less than 30
Easy bruising
Petechiae,purpura
Mucosalbleeding
Severebleedingaftertrauma
Intracranialhaemorrhage
difference in clinical presentation in Hemohilia and VWF disease
there is no joint bleeding in Vwf
Explain the differences between
thrombocytopenia caused by marrow failure and thrombocytopenia caused by peripheral destruction
A 70 year old man suffered from severe abdominal pain and rapidly developed shock (low blood pressure and rapid pulse). He was admitted to hospital but, unfortunately, despite emergency surgery he died.
What process has occurred?
Why is the intestine this colour?
Why is he in shock?
Haemorrhagic (red) infarction
Because numerous vascular anastomoses are present within the small bowel. Occlusion of the main blood supply causes an infarct. The collateral arterial supply is insufficient to rescue the tissue but does allow blood to enter the dead tissue creating a red infarct.
damaged bowel wall> becomes ‘leaky’ and toxic substances (such as the gram - bacterial product endotoxin) enter the circulation.
This results in massive activation of inflammation, widespread vasodilatation, loss of fluid from the vascular spaces into the extracellular space and therefore shock.
warfarin
mechanism
cjlinical use
The EX-President went to WAR farin
