Disorders of Haemostasis Flashcards
What are exampleds of minor bleeding symptoms?
How often do they occur?
Many are very common:
Easy bruising 12%
Gum bleeding 7%
Frequent nosebleeds 5%
Bleeding after tooth extraction 2.5%
Post operative bleeding
Family histroy 44%
What are expamples for a heavy bleeding histroy?
- Epsitaxis >10min
- bruising on several sites
- heavy menstrual bleeding
- prolonged bleeding after minor injury >10min
- etc.
What are possible reason for thrombocytopaenia
Insuficcient production
- Leukaemia
- Megaloblastic anaemia (B12 deficiency)
- Bone Marrow failure
Or too much clearing/break down
- Immune (ITP) (shortened half life)
- DIC (pull in spleen)
What is auto-ITP?
Auto-immune thrombocytopenic purpura
–> antibodies produced that bind to the platelets and triggers phagocytosis by macrophages
What are the possible disfunctions of platelets that might lead to a impaired function
- e.g. Hereditary absence of glycoproteins or storage granules
- Aquired: Drug abuse (ibuprofen, ASS)
What is VWBD?
Normally inherited
- low number of VWBF abnormal structure of VWBF
- Type 1+3: too less
- Type 2: abnormal function
What are the functions of the VWBF?
- connection of platelets and collagen
- stabilisation of factor VIII (may be low when VWBF is very low)
What are aquired and inherited disorders of the vessel wall that might influence haemostasis?
Inherited (rare) –> Ehlers Danlos syndrome (hyper mobile skin)
Aquired: scurvy, steroid therapy, ageing, vasculitis
What is the typical signs and symptoms in problems in primary haemostaiss?
Don’t stop bleeding:
- epistaxis, prolonged bleeding, gum bleeding, mucosal bleeding (gum, menstrual), bruises
- prolonged bleeding after trauma/injury
In thrombocytopenia: Petichae
Severe VWD: haemophilia-like bleeding
What are petichae?
What is the underlying reason?
Low platelet count
How could you test for problems in primary haemostasis?
- Platelet count, platelet morphology
- Bleeding time (PFA100 in lab)
- Assays of von Willebrand Factor
- Clinical observation
What are conditions in which there are deficiencies in coagulation factors
- Haemophilia A(Factor VIII) B (IX)
- severe but compatible with life
- spontaneous joint and muscle bleeding
- Prothrombin (II)
- not compatible with life
- Factor XI –> excess bleeding
- XII –>. no excess bleeding at all
–> all different severity
What are the hereditary and aquired problema in secondary haemostasis
Decreased production
- Inherited:
- Haemophilia A+B
- Aquired: much more common
- liver disease
- dillution (blood transfusion wihtout plasma)
- drugs (e.g. warfarin, heparin)
Increased consumption (aquired)
- Disseminated intravascular coagulation (DIC)
- Immune - autoantibodies
What is DIC?
Disseminated intravascular coagulation (e.g. in cancer/ sepsis)
- increased consumption of clotting factors in circulation
- via increased TF in bloodstream–> increased, generalised coagulation
- Fibrin cloths forming in organs together with bleeding because platelets and anti-coagulants are all used
How do people with a problem in secondary haemostasis presnet?
- no presentation with small injuries (e.g. small vessels)
- common bruising (rare nosebleed)
- bleeding into joints/muscels (haemophilia)
- frequent restart and stopping of bleeding
- pbleeding after trauma may be delayed and is prolonged
Which test could you perform to test for coagulation disorders? (secondary)
- Screening tests (‘clotting screen’)
- Prothrombin time (PT) (VII, X, V, II)
- Activated partial thromboplastin time (APTT) (intrinsic system XII, XI , VIII, X. V, II)
- –> Components of plotting screen
- Full blood count (platelets)
Factor assays (for Factor VIII etc)
Tests for inhibitors
