Summary an approach to thrombotic thrombocytopenic purpura

THROMBOTIC THROMBOCYTIC PURPURA & RELATED
THROMBOTIC MICROANGIOPATHIES

By Shafika Nabila

,
, DEFINITION OF TTP

• Is a Thrombotic microangiopathy (TMA) characterized clinically by
presence of microangiopathic haemolytic anaemia (MAHA) and
thrombocytopenia with pathological features including occlusive
microvascular/Macrovascular disease .
• Life threatening medical emergency
• Untreated TTP has high mortality due to sudden neurological and
cardiac dysfunction .




British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies” British
Journal of Haematology (2023).

, PATHOGENESIS
• Caused by severe
deficiency of
metalloproteinase
ADAMTS13
• ADAMTS13 is synthesized
in the liver and cleaves
von Willibrand factor
(VWF)
• In ADAMTS13 Deficiency
results in platelet- rich
thrombi in the
microvasculature, red
cell
fragmentation/heamolysis
British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic
microangiopathies” British Journal of Haematology (2023).