Bleeding disorders Flashcards
(35 cards)
thrombocytopenia causes
purpura and bleeding from mucous membranes
immune thrombocytopenic purpura (ITP) =
immune destruction of platelets
how does ITP present in children
acute onset mucotaneous bleeding often recent history of viral infection
viral infections precipitating ITP in children
Varicella zoster/ measles, glandular fever
ITP presentation in adults
less acute,
seen more in women
associated with other autoimmune conditions
clinical features of ITP
easy bruising, purpura, epistaxis, menorrhagia
investigation results in ITP
platelet auto-Ig
thrombocytopenia
treatment of ITP
- steroids and Iv IgG and anti-D affective in children
- steroids or splenectomy in adults
thrombocytic thrombocytopenia purpura (TTP) =
platelet consumption leads to profound thrombocytopenia
characteristics of TTP
- fever,
- cerebral dysfunction
- microangiopathic haemolytic anaemia
- RBC fragmentation
- AKI
coagulation screen in TTP
normal, raised LDH due to haemolysis
why does TTP occur
ADAMTS-13 reduction causing endothelial damage and microvascular thrombosis
what does ADAMTS-13 normally down-regulate
vWF
treatment of TTP
plasma exchange
corticosteroids + rituximab
Prothrombin time measures which factors
7, 10, 5, PR and fibrinogen
activated partial thromboplastin time measures
12, 11, 9, 8, 10, 5
bleeding time measures
platelet plug formaiton
Haemophillia A is due to
lack of factor 8
extremely low factor 8 leads to
frequently spontaneous bleeding early in life into tissues and muscles -crippling
moderate level reduction in factor 8 leads to
severe bleeding following injury and occasional spontaneous
mild reduction in factor 8 causes
excess bleeding after injury or surgery
treatment of Haemophillia a
- Iv factor 8 concentrate PRN
- desmopressin if mild
Haemophillia B due to
lack of factor 9
which is more common haemophillia a or b
a
