Thrombocytopenia

Question 1

What is Thrombotic thrombocytopenic purpura (TTP)?

Answer 1

Rare life-threatening blood disorder
ADAMTS13 deficiency so cannot cleave von Willebrand factors
Multimeters of VWF cause platelets to clump in vessels
Causing thrombocytopenia (bleeding and organ failure)
Clots in small vessels break up RBCS = haemolytic anaemia
EMERGENCY

Question 2

How does thrombotic thrombocytopenic purpura (TTP) present?

Answer 2

Adult females
Thrombocytopenia
Fever
Headaches
Purpura
Renal failure
Fatigue
Abdominal pain
Neurological issues: confusion/ partial paralysis/mental changes/speech abnormalities

Question 3

How do you investigate thrombotic thrombocytopenic purpura (TTP)?

Answer 3

Thrombocytopenia on bloods
Blood film (Schistocytes)
Further tests to exclude Ddx (aplastic anaemia, leukaemia)

Question 4

How to mange a patient with thrombotic thrombocytopenic purpura (TTP) ?

Answer 4

Plasma exchange with FFP
Steroids for persistent cases
Rituximab (monoclonal antibody against B cells)
Escalate to senior

Question 5

What is the prognosis for thrombotic thrombocytopenic purpura (TTP)?

Answer 5

Response is good to plasma
Mortality rate can be 10-20%

Question 6

What is Immune thrombocytopenia purpura (ITP)?

Answer 6

Idiopathic Autoimmune disease where thrombocytopenia occurs

Question 7

How does Immune thrombocytopenia purpura (ITP) present?

Answer 7

Can affects anyone
Thrombocytopenia
systemically well
- petechiae (non-blanching red spots)
- bleeding from gums
- purpura
- concerning signs of bleeding (persistent headaches and melaena
- viral infection associated

Question 8

How to investigate immune thrombocytopenia purpura (ITP)?

Answer 8

Reduced platelets on FBCs

Question 9

How to manage Immune thrombocytopenia purpura (ITP)?

Answer 9

Supportive
Prednisolone and PPI
IV IG (acute bleeding)
Thrombpoetien analogues
splenectomy (last resort, if no response to treatment)

Question 10

What is Heparin Induced Thrombocytopenia?

Answer 10

Development of heparin induced antibodies (HIT) Against platelets when exposed to heparin

Question 11

How to manage Heparin Induced Thrombocytopenia?

Answer 11

Test for HIT antibodies
Stop heparin and use alternative anticoagulant

Question 12

What causes a deficiency in ADAMTS13 enzyme in TTP?

Answer 12

Genetic mutation or autoimmune attack against the enzyme