Working Problem 10-Thrombocythemia Flashcards
What does physical examination reveal in Von willebrand disease?
Bruises
Gum bleeding
menorrhagia
How can vitamin C deficiency cause bleeding?
It can result in scurvy where the vascular collagens are not formed properly leading to bleeding
It leads to gum bleeds and is a type of acquired VASCULAR disorder
Not serious
What is seen in the basic and detailed coagulation studies in VWD
Everything normal except for VWF,factor 8,RCoF and cofactor assay
What is the most common inherited Coagulation disorder and how is it inherited?
VWD
it is autosomal dominant at chromosome 12 with variable penetrance
What are the types of VWD are there?
Type 1 –partial reduction (20-50%)in the level of otherwise normal vWF(commonest)
Type 3-complete absence of vWF
Type 2-abnormal vWF or abnormal binding
•Mild bleeding disorder
Type 2A-loss of high molecular weight vWF multimers
Type 2B-vWF has abnormally high affinity for platelets
Type 2M-vWF with reduced affinity for platelets or Subendothelium
Type 2N-vWF with reduced affinity for factor 8
Clinical Features of VWD?
- Mucosal bleeding(epistaxis,gums,GIT,menorrhagia)
- Increased bleeding from cuts and abrasions
- Increased haemorrhage with surgery and trauma
- Haemarthrosis and muscle haematomas are rare except in type 3 disease
Management of VWD
- Tranexamic acid
- Desmopressin(DDAVP )
- vWF replacement(factor 8 replacement)
What are the type of Hemophilia present?
Haemophilia
A→factor 8 deficiency
B→factor 9 deficiency
What are the causes of Hemophillia
• both are x linked recessive inheritance
1/3 of them due to spontaneous mutation
What are the clinical manifestation of hemophilia?
What is the distinguishing factor from VWD?
Dependent on severity of disease
can cause spontaneous bleeding and joint crippling deformity(Due to hemarthroma) and can be as small like post traumatic bleeding
Distinguishing feature hemathroma
Laboratory finding for Hemophllia
aPTT prolonged
Factor 8 and 9 in the clotting assay abnormal
How do you manage hemophilia?
Tranexamic acid
Desmopressin(Increase vWF to confer more protection for factor 8)
Porcine factor 8
recombinant factor 8
What are the laboratory findings in vitamin K deficiency
• Vitamin K dependent coagulation factors are 2,7,9 and 10
• Extrinsic pathways(factor 2,7 and 10)
prolongation of Prothrombin time
- Intrinsic pathway (factors 9,10 and 2)
- prolongation of activated partial thromboplastin time(aPTT)
What are the sources of vitamin K?
K1 from animal source and vegetables
K2 from bacteria in gut
It is fat souluble
Management of Vitamin K deficiency
Same as how you will reverse warfarin
Active bleeding
• replace the coagulation factors with an IV infusion of either fresh frozen plasma(FFP) or prothrominex
• Correct vitamin K deficiency by with Vit K1,10mg by subcutaneous injection
Prophylaxis
a. Neonate: give a single vit K1 injection 1 mg at birth
b. Adult :give vit K1,
10 mg orally on daily basis for as long as necessary
