thrombotic thrombocytopenic purpura

Question 1

Define thrombotic thrombocytopenic purpura (TTP)

Answer 1

Clinical syndrome characterised by microangiopathic heamolytic anaemia and thrombocytopenic purpura

other signs form the TTP pentad, but not always there -neuro dysf, renal dysf, fever

very fatal if untreated

Question 2

Aetiology and risk factors of thrombotic thrombocytopenic purpura

Answer 2

Unclear, but can be due to production of large VWF-form platelet aggregates-deplete platelets and form microvascular thrombi-which causes rest of Sx
usually an aditional stressor agent is needed to initiate the TTP
Genetic cause-lack of enzyme to cleave vWF

can also be aquired-believed to be 2ndary to autoimmune

Risk factors
Women > men
Black people
genetic-present around 30
Obese, pregnant

Question 3

Epidiemology of thrombotic thrombocytopenic purpura

Answer 3

Very rare-2.2 in 1 million in UK

Question 4

Signs and sx of thrombotic thrombocytopenic purpura

Answer 4

Its the pentad
Hx also usually has a something causing physio stress about 1 week before

Microangiopathic heamolytic anemia (pallor, fatigue, SoB), jaudice, bleeding

Thrombocytopenia-purpura, bruising, bullae, menrragia

Fever

Neuro Sx–severe-coma, seizures, focal/localised issues -stroke like
Milder-headache, confusion

Digestive (GI thrombus)-nausea, vomiting, diarrhoea, abdo pain)

Renal infovlement-similar to Heamolytic uraemic syndrome
Urea and creat-severe renal fail in 5 % of pt-raised

Question 5

Investigations of thrombotic thrombocytopenic purpura

Answer 5

FBC-microcytic anemia-VERY low
Thrombocytopenia
haptoglobin-low In heamolytic aneamia

Smear-shicstocytes
Reticulocytes-raised
Urinalysis-proteinuria
Urea and creat-severe renal fail in 5 % of pt-raised

Coombs test-for autoimmune heamolytic aneamia-negative