Haematology Flashcards
Coagulation disorder suspected but coagulation profile is normal - think of
Factor XIII deficiency
What does factor XIII do?
Makes crosslinks between fibrin dimers, so stabilises the clot
What kind of bleeding do you get with factor XIII deficiency?
Bleeding a few days after the injury
How is factor XIII deficiency inherited?
Autosomal recessive
How is factor XIII deficiency managed?
Either with factor XIII supplementation or cryoprecipitate infusions
Which is the most severe vWF disease?
Type 3 (autosomal recessive)
What does von willebrand factor do?
Acts as the transporter protein for factor VII and also helps platelet binding at endothelial sites
How do you test for vWF?
Partial thromboplastin time often prolonged; ristocetin cofactor activity test measures plasma vWF function
What are the coagulation screen abnormalities in factor XII deficiency?
Prolonged partial thromboplastin time
How is factor XII deficiency inherited?
Autosomal recessive
What causes allergic transfusion reactions?
Interaction between donor plasma proteins and recipient IgE antibodies
What haematological abnormality does phenytoin cause?
Folate deficiency -> macrocytosis
What haematological abnormality does hydroxyurea cause?
Macrocytosis with megaloblastic anaemia
What haematological abnormality can penicillin cause?
Haemolytic anaemia
What haematological abnormality can cotrimoxazole cause?
Aplastic anaemia
Which disease: pancytopenia, short stature, cafe au lait spots, squint, limb anomalies, mental retardation, chromosome fragility, predisposition to leukaemia
Fanconi anaemia
How is Fanconi anaemia inherited?
Autosomal recessive
What haematological abnormalities are seen in Diamond-Blackfan syndrome?
Normo- or microcytic anaemia with low reticulocyte count
Iron studies in iron deficiency anaemia
Low serum iron High TIBC Decrease in transferrin saturation Increase in serum soluble transferrin receptors Low ferritin Increased zinc protoporphyrin/haem ratio Decreased serum ferritin