Bleeding Disorders Flashcards
How do coagulation disorders present?
Bleeding into deep tissues, muscles, joints; delayed but severe bleeding after injury (often prolonged)
How do vascular defects present?
Superficial bleeding into skin/mucosal membranes = petechia, purpura, easy bruising ; bleeding immediately after injury
How do platelet disorders present?
Superficial bleeding into skin/mucosal membranes; bleeding immediately after injury
Name some congenital causes of vascular defects?
Osler-Weber-Rendu Syndrome, Ehlers-Danlos Syndrome, Marfans, Pseudoxanthoma elasticum, Osteogenesis Imperfectaother CTDs
What are the main causes of acquired vascular defects?
Senile purpura, infection, steroids, scurvy
Name 3 infections that commonly present with vascular defects
Measles, Dengue fever, Meningococcal infections
What vascular presentation is seen in scurvy?
Perifollicular haemorrhages
What is another name for Osler-Weber-Rendu Syndrome?
Hereditary Haemmorhagic Telengiectasia
Bleeding disorders are split into 4 groups, based on their aetiology. List these groups.
Vascular defects (easy bruising), Platelet disorders (low or abnormal function), coagulation disorders (factor deficiency), mixed
What is the inheritance pattern of Osler-Weber-Rendu Syndrome? What 2 genes are commonly affected?
Autosomal Dominant; ENG, ALK1
What is the diagnostic criteria for OWR Syndrome?
Clinical diagsnosis. 3/4: epistaxis, telengiectasia, visceral lesions, FHx
What happen in OWR syndrome/HHT?
Vascular dysplasia (mucocutaneous telengiectasia, arteriovenous malformation) causes increased bleeding
What is the definition of Thrombocytopenia?
Platele count under 150 bill g/L
What are the acquired casues of decreased platelet function?
Aspirin, Cardiopulmonary bypass, Uraemia
What are the congenital causes of decreased platelet function?
Storage pool disease, Thrombasthenia (glycopprotein deficiency)
Causes of thrombocytopenia?
Low Production: bone marrow failure
Increased destruction: AITP, ITP, Drugs (eg Heparin), DIC, HUS, TTP
Epidemiology of Acute ITP
Children (2-6y), F:M 1:1
Aetiology of Acute ITP
Most commonly preceded by an infection
How quickly does Acute ITP present and how long does it last?
Abrupt onset, lasts 2-6 weeks
Treatment of Acute ITP?
Normally self limiting - spontaneous remission
Epidemiology of Chronic ITP
Adults; F:M 3:1
How quickly does Chronic ITP present and how long does it last?
Symptoms can present abruptly or indolently; symptoms are normally long term
Aetiology of Chronic ITP?
Associated with Autoimmune Disease, CLL and HIV
Treatment of Chronic ITP
IVIg, steroids, splenectomy
What are the 3 main inherited coagulation disorders?
Haemophilia A, Haemophilia B, von Willebrand’s disease
What are the 3 main acquired causes Coagulation disorders?
DIC, Liver Disease, Vit K deficiency
What factor is deficient in Haemophilia A, Haemophilia B, vW Disease?
Haemophilia A = Factor VIII, Haemophilia B = Factor IX, von Willebrand’s disease = Factor VIII (also decreased platelet function)
What is another name for Haemophilia B?
Christmas Disease
Inheritance pattern of Haemophilia A?
X linked recessive
Inheritance pattern of Haemophilia B?
X linked recessive
Is Haemophilia A or B more common?
A = 1/10000 males (B=1/50000)
How is vW Disease most commonly inherited?
Autosomal Dominant
Mgmt for Haemophilia A
Avoid NSAIDs and IM injections, desmopressin, lifelong factor VIII rconcentrates
Mgmt for Haemophilia B
Factor IX concentrates
Mgmt for VW Disease
Desmopressin, VWF and Factor VIII concentrates
What factors require Vit K for synthesis?
2, 7, 9, 10
Which factors have impaired synthesis in Liver disease?
What else is affected?
Decreased synthesis of Factors of 2, 7, 9, 10, 11, and fibrinogen
Decreased absorption of Vit K
Abnormalities of platelet function
Treatment for Vit K deficiency
IV vitamin K or FFP for acute haemorrhage
What is the platelet count at presentation for Acute ITP and Chronic ITP?
Acute ITP = under 20,000 Chronic ITP = under 50,000
How is Haemophilia graded? Define each grade.
Related to factor level: Severe = under 1%, moderate = 1-5%; mild = 5-25%
How does Haemophilia (A&B) present clinically?
Often present early in life or with prolonged bleeding after surgery/trauma
How does vW disease present clincally?
often bleeding indicative of platelet disroders (i.e mucocutaneous bleeding) but can also include bleeding indicative of coagulation disorders
What are the causes of Vitamin K Deficiency?
Vitamin K malabsorption/malnutrition, Warfarin, Abx therapy, biliary obstruction
What are the causes of DIC?
Malignancy, sepsis, trauma, obstetric complication, toxins
Treatment for DIC
Treat the cause, give transfusions, FFP, platelets, cryo etc.
What happens in DIC?
Widespread activation of coagulation causes clotting factors and platelets to be consumed which then causes increased risk of bleeding
Clotting Results for Heparin: INR, APTT, TT, Platelet Count, Bleeding Time
INR - high APTT - very high TT - very high Platelet Count - normal Bleeding Time - normal
Clotting Results for DIC: INR, APTT, TT, Platelet Count, Bleeding Time
INR - very high APTT - very high TT - very high Platelet Count - low Bleeding Time - high Also high D-dimer
Clotting Results for Liver Disease: INR, APTT, TT, Platelet Count, Bleeding Time
INR - high APTT - high TT - normal or high Platelet Count - normal or down Bleeding Time - normal or high Also high AST
Clotting Results for Platelet Defect: INR, APTT, TT, Platelet Count, Bleeding Time
INR - normal APTT - normal TT - normal Platelet Count - normal Bleeding Time - increase
Clotting Results for Vit K Deficiency: INR, APTT, TT, Platelet Count, Bleeding Time
INR - very high APTT - high TT - normal Platelet Count - normal Bleeding Time - normal
Clotting Results for Haemophilia: INR, APTT, TT, Platelet Count, Bleeding Time
INR - normal APTT - very high TT - normal Platelet Count - normal Bleeding Time - normal
Clotting Results for Von Willebrand’s Disease: INR, APTT, TT, Platelet Count, Bleeding Time
INR - normal APTT - very high TT - normal Platelet Count - normal Bleeding Time - high
