3: Bleeding Disorders Flashcards
What is a bleeding disorder
abnormal Haemostasis that can result in diathesis
What coagulation pathway does PT look at and how is that remembered
Extrinsic
Personal trainers focus on extrinsic appearance
What factors comprise the extrinsic pathway
TF, 7, 10, 5, 2, Fibrinogen
What disorders can cause only PT to be raised
- Heparin
- Von Wille Brand Disease
What coagulation pathway does the APTT look at
intrinsic
What factors make up the intrinsic pathway
12 11 10 9 8 5 2
What 3 disorders cause raised isolated APTT
- Haemophilia A
- Haemophilia B
- Antiphospholipid syndrome
What causes raised APTT and PT
DIC
Liver disease
Vitamin K deficiency
What factors are vitamin K dependent
1972
What is the inheritance pattern of haemophilia A
X-Linked recessive
What factor does haemophilia A affect
F8
Is haemophilia A or B more common
Haemophilia A (80%)
Explain presentation of haemophilia A
- Haemoarthrosis (bleeding into joints)
- Bleeding into muscle
- Bleeding into soft tissue
What does petechial bleeding indicate
Platelet deficiency - not clotting factor
What will be seen on coagulation studies in haemophilia A
Prolonged APTT
What is diagnostic of haemophilia A
Factor 8 assay
What should be avoided in patients with haemophilia A and why
IM injections and NSAIDs - can lead to proloneed bleeding
If a patient has mild haemophilia A what is given
Control bleeding with pressure and elevation
Desmopressin can increase F8
what drug is given in mild haemophilia A to increase F8
Desmopressin
what is given for major bleeds
Recombinant F8 transfusion
what is haemophilia B also called
Christmas disease
what is the inheritance pattern of haemophilia B
X-linked recessive
what factor is deficient in haemophilia B
F9
what is more common haemophilia A or B
Haemophilia A (90%)
how does haemophilia B present
- bleeding in JOINTS, MUSCLE AND SOFT-TISSUE
- may present with epistaxis or excessive bleeding post-procedure
what will be seen on coagulation studies in haemophilia B
Prolonged APTT
what is diagnostic of haemophilia B
F9 assay
how is haemophilia B managed
recombinant factor 9
what is the most common inherited bleeding disorder
Von Wille Brand Disease
what is the role of VWF
When tissue is damaged it exposes VWF which causes platelet aggregation. VWF also stabilises F8 in the coagulation cascade.
Where is VWF found
Weibel-Palade bodies of endothelial cells
What is von wille brand disease
deficiency of VWF
What is the inheritance pattern of von wille brand disease
Autosomal dominant
What does VWD lead to
deficiency of VWF and F8
Explain symptoms of VWD
behaves as a platelet disorder causing epistaxis and menorrhagia.
opposed to symptoms of haemoarthrosis and bleeding into joints which are very rare
What investigation is ordered in VWD
- Coagulation studies
- F8 assay
What will be seen on coagulation studies
Prolonged APTT and PT
How is bleeding in von wille brand disease managed
Tranexamic acid
What medication can be given in von wille brand disease
Desmopressin - to increase VWF
