haemostasis and anaemia Flashcards
What is normal haemostasis?
- Localised vasoconstriction at injury site
- Platelet adhesion to vessel wall and formation of platelet aggregation/plug
- Coagulation cascade activation—> fibrin formation (reinforces plug)
- Fibrinolytic system activation which digests plug, reestablishing vascular patency
What are platelets?
Small non-nucleated cells Fragments of megakaryocyte cytoplasm 140-350 x10^9/l <20- spontaneous bleeding >80- haemostatic Thrombocytopenia- reduced Thrombocytosis
What is primary haemostasis?
Present w purpura, bruising, bleeding from cuts, heavy periods, surgical bleeding
~inv vessel wall, platelets and Von Willebrand factor
What is secondary haemostasis?
Present w joint and muscle haematomas and surgical bleeding, bruising not prominent
~inv coagulation cascade
How should a patient w a bleeding disorder be managed?
History- bruising? bleeding after minor injury/extractions/surgery? epistaxis? menorrhagia? family history? drugs (eg. aspirin, warfarin, cytotoxics)? alcohol?
Examination- ecchymoses? purpura? haemarthrosis? damaged joints? anaemia? signs of liver disease? splenomegaly?
Investigations- full blood count? clotting screen (PT, APTT, fibrinogen)? bleeding time/PFA?
Treatment- depends on diagnoses Haemophilia A- FVIII concentrate Haemophilia B- FIX concentrate Thrombocytopenia- platelet transfusion Warfarin overdose- Vit K
What are some bleeding terms?
Menorrhagia- heavy menstrual periods Epistaxis- nose bleed Haemoptysis- coughing blood Haematemesis- vomiting blood Haematuria- blood in urine Malaena- black stool (bleed in upper GIT) Ecchymoses- bruises Purpura- purple blood spots
What does PFA stand for?
Automated platelet function analysis
What are some inherited vascular bleeding disorders?
Hereditary haemorrhagic telangiectasia
Ehlers-Danlos syndrome
What is Ehlers-Danlos syndrome?
V flexible joints
Stretchy and fragile skin
What is hereditary haemorrhagic telangiectasia?
Arteriovenous malformations
Regular nosebleeds
Red/purple spots/lines under skin
What are coagulation factor disorders?
Haemophilia A and Haemophilia B= x linked
Von Willebrand disease- autosomal dominant
Others- autosomal recessive, deficiciency of fibrinogen or factors II, V, VII, X, XI, XIII
What is the frequency of bleeding disorders?
Haemophilia A- 1:5000
Haemophilia B- 1:25000
VWD- 1:1000
What is Haemophilia?
A- factor VIII deficiency
B- factor IX deficiency
Identical clinical features
Sex linked inheritance
Sites of bleeding- joints, muscles, post trauma, post op
Severity- <1%- severe 1-5%- moderate 5-50%- mild 50%-150%- normal
How is haemophilia treated?
Mild Haem A- desmopressin (releases factor VIII from endothelium)
All severities- factor VIII/IX concentrate- UK recombinant products
Plasma derived products- risk of infection, all products virally inactivated since 1985
Haemophiliacs treated before 1985 (HIV 30%, Hep B 5%, Hep C 98%)
What is Von Willebrand Disease?
Autosomal dominant
3 main types (many subtypes)
Milder than haemophilia
Clotting factor + platelet abnormality
Sites of bleeding- bruising, cuts, gums, epistaxis, menorrhagia, post op, post trauma
Treatment-
~desmopressin (DDAVP)
~intermediate purity (plasma derived) factor VIII
What tests should be done for Haem A?
Factor VIII- low
APTT- prolonged
What tests should be done for Haem B?
Factor IX- low
APTT- prolonged
What tests should be done for VWD?
Factor VIII- low
VW factor- low
BT- Prolonged
APTT- Prolonged
What are some inherited platelet disorders?
Affecting number-
MYH-9 related thrombocytopenia
32 other inherited causes of it
Affecting function-
Glanzmann’s thrombasthenia
Storage pool disease
What is thrombosis?
Blood coagulation in a vessel
Arterial-
~high pressure, platelet rich
~eg. MI, stroke
~treat- anti platelet drugs
Venous-
~low pressure, fibrin rich
~eg. deep vein thrombosis, pulmonary embolism
~treat- anticoagulants
What are risk factors for venous thrombosis?
Acquired-
~age, previous VTE, surgery/trauma, immobility, obesity, cancer/serious illness, pregnancy, the pill, hormone replacement therapy
Inherited thrombophilia-
~antithrombin deficiency (1:3000), protein C deficiency (1:300), protein S deficiency (1:300), factor V Leiden mutation (1:20), prothrombin G20210A condition (1:75)
What are risk factors for arterial thrombosis?
Smoking, obesity, hypertension, diabetes mellitus, older age
Not inherited
What are some aquired vascular bleeding disorders?
Senile purpura Scurvy (Vit C deficiency) Steroid purpura (long term use)
What are causes of thrombocytopenia?
Failure to produce-
~Vit B12 or folate deficiency
~disease infiltrates bone marrow
~radiation, cytotoxic therapy
Increased consumption-
~immune thrombocytopenia
~disseminated intra vascular coagulation
~HIV infection
Hypersplenism