Bleeding disorders Flashcards
What is a normal platelet count?
150-400
What is ITP?
Idiopathic thrombocytopenic purpura
- An autoimmune condition in which antibodies are produced against platelets , can develop in adults or children.
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What do the bloods show in ITP?
Bleeding time increased.
Low platelets.
Bone marrow biopsy = increased megakaryocytes.
Management of ITP in adults and children?
Adults usually present with epistaxis and menorrhagia.
- EASY BRUISING.
Treatment only given if severe: 1st = steroids, 2nd = splenectomy.
Children usually present with mild mucosal bleeding; usually self limiting and does not require management.
What is thrombotic thrombocytopenic purpura?
Haematological emergency characterised by low platelets and MAHA (microangipathic haemolytic anaemia)
How does TTP present?
Presents with fever, mucosal bleeding, AKI with haematuria and proteinuria,
Fluctuating CNS signs are also common, drowisness, confusion, seizures.
What is the mutation in TTP?
ADAMST-13
What does the blood film show in TTP?
Schistocytes.
Management of TTP?
Plasmaphoroessis, steroids and immunoglobulin.
What is haemolytic uraemia syndrome associated with?
Associated with ECOLI 0157
Bloody diarrhoea followed by oliguria/haematuria.
How are the haemophiliac inherited?
X linked recessive.
Haemophilia A?
Factor 8 deficiency
Haemophilia B?
Factor 9 deficiency (Christmas disease)
Von Willebrands inheritance?
Autosomal dominant
What do the blood show in Von Willebrands disease?
Low VWF, Low factor VIII, High APTT.
Von willebrands circulates bound to factor 8. Factor 8 only survives a short time without VWF. Therefore if no VWF present, low levels of factor VIII will be present.
