primary haemostatic failure

Question 1

how do patients with VWF disease present?

Answer 1

family history (AD)
signs of thrombocytopenia - low platelets = heavy menstrual bleeds, nosebleeds etc

Question 2

what will be seen on investigation of VWF disease?

Answer 2

prolonged APTT
normal platelets

Question 3

what is the management for VWF disease?

Answer 3

tranexamic acid (mild bleeding)
desmopression (raised VWF levels)

Question 4

how would a patient with ITP usually present?

Answer 4

child following infection

Question 5

what will be seen on investigation?

Answer 5

thrombocytopenia - low platelets -> bleeding

Question 6

what is the management of ITP?

Answer 6

steroids, IVIG
rituximab, splenectomy

Question 7

how will a patient with TTP present?

Answer 7

fever
fluctuating CNS

Question 8

what is the main signs/symptoms to look for in a question on TTP?

Answer 8

fever
microangioplastic anaemia - low Hb and signs of haemolysis
renal failure - raised creatinine
neuro effects - confusion, headaches

Question 9

what will be seen on investigations of TTP?

Answer 9

thrombocytopenia
schistocytes

Question 10

what is the management for TTP?

Answer 10

plasma exchange therapy

Question 11

how would a patient with haemolytic uraemic syndrome usually present?

Answer 11

child with diarrhoea
acute kidney injury

Question 12

what will be seen on investigation of HUS?

Answer 12

thrombocytopenia

Question 13

what is the management for HUS?

Answer 13

supportive, dialysis + ecluzimab