Bleeding disorders Flashcards
what are the clinical features of haemophilia?
haemarthrosis muscle haematoma CNS bleeding retroperitoneal bleeding post surgical bleeding
what are the complications of haemophilia?
stroke
neuromuscular compression (compartment syndrome)
synovitis
chronic haemophilic arthropathy (due to repeated haemarthrosis)
what factors are reduced in haemophilia?
factor VIII or IX
8 or 9
what tests would you carry out to investigate haemophilia?
APTT
prothrombin
factor VIII and factor IX
genetic analysis
what would the APTT and prothrombin test show in someone with haemophilia?
APTT increased/prolonged
prothrombin normal
how is bleeding in haemophilia treated?
coagulation factor replacement (factor VIII or IX)
tranexamic acid
desmopressin (DDAVP)
how can chronic haemophilic arthropathy be treated?
synovectomy
joint replacement
what are the complications of desmopressin (DDAVP) ?
MI
hyponatraemia (babies)
what type of bleeding occurs in von willebrands disease?
platelet type bleeding (mucosal)
what is the pattern of inheritance in haemophilia and von willibrands?
haemophilia is X linked
von iwllebrands is autosomal
what is the management options for von willebrands disease?
vWF concentrate or desmopressin (DDAVP)
transexamic acid
topical applications
OCP
what are the causes of thrombocytopenia?
decreased production;
- marrow failure
- aplasia
- infiltration
increased consumption;
- immune ITP
- non immune DIC
- hypersplenism
what is the clinical presentation of thrombocytopenia?
petechiae
ecchymosis
mucosal bleeding
rare CNS bleeding
what is immune thrombocytopenia associated with?
infections i.e. EBV, HIV
collagenosis
lymphoma
drug induced
what is the classical presentation of a coagulation factor disorder?
articular bleeding - haemarthrosis
muscle haematoma
CNS bleed i.e intracranial haemorrhage
what is the classical presentation of a platelet type bleeding disorder?
mucosal epistaxis purpura menorrhagia GI bleed
what is the difference in the phenotype of factor VIII and IX haemophilia?
no diference
identical phenotypes
don’t know which one you have until you test the factor
what is the most common joint to be affected by haemarthoris sin haemophilia?
ankle
what’s the difference between haemophilia A and haemophilia B?
A - absence factor VIII
B - absent factor IX
what is the treatment for idiopathic thrombocytopenia?
steroids
IV IgG
splenectomy
thrombopoeitin analogues i.e. eltrombopag and romiplostin
what prophylaxis is given to prevent haemorhagic disease of the newborn?
vitamin K injection
what can put a baby at risk of haemorrhage disease of the newborn?
vitamin k deficient diet esp breast
immature coagulation systems
give examples of acquired bleeding disorders.
thrombocytopenia liver failure renal failure DIC drugs - warfarin, harping, aspirin etc
what’s the difference between type 1, 2 and 3 von willebrands disease?
type 1 = quantitative (don’t have enough vWF)
type 2 = qualitative defect (abnormal vWF and extent is determined where it occurs on the vWF gene)
type 3 = complete deficiency
when assessing a patient for possible bleeding disorder, what are the main questions you have to ask in the history?
does this patient have a bleeding disorder?
- previous surgeries i.e. tooth extraction? post op bleeding?
how severe? - mild trauma or spontaneous bleeding
pattern of bleeding - platelet or coagulation?
acquired or inherited - age at first presentation? FX? previous episodes?
what is NAIT?
neonatal alloimmunity thrombocytopenia
- a baby is born with a low platelet count due to the mothers own immune system attacking the babies platelets
what is the typical presentation of a baby is NAIT?
purpura
thrombocytopenia
