Académique Documents
Professionnel Documents
Culture Documents
thrombocytopenic purpura :
Current Perspective
Rangga Lunesia
Abstract
TTP
Rare ( 6 cases/mill/y in UK)
Life threatening
Underlying path: def. ADAMTS13
Introduction
TTP
Aggressive form of thrombotic
multimer to smaller
vWF as bridge between exposed
subendothelial matrix+platelet, after vessel
injury
Vessel injuryEndothelial cells secreted
ULvWF multimer
<< ADAMTS13 activity >> ULvWF multimer
excession bind to platelet (platelet rich
thrombi) platelet aggregation partial
vessel occlusion organ ischaemia
function (95%)/Acquired
Primary : no overt relation to immune dysregulation
Secondary : overt relation immune dysregulation
(e.g. : SLE,HIV)
(5%)/Congenital
Diagnosis
Clinical Presentation
Classic pentad : fever, thrombocytopenia,
Laboratory assays
<< ADAMTS13 enzyme activity(<10%)
fibrinogen
Liver function test, electrolytes, urea and serum creatinine,
serum troponin, calcium, thyroid function test
HIV serology, hepatitis B and C serology
Autoantibody screen
Pregnancy testing
Direct antiglobulin test, cytomegalovirus serology
CT/MRI brain, echocardiogram, EKG
Other imaging to exclude malignancy
Management of TTP
ACQUIRED TTP
Clinical
Plasma Exchange(PEX)
Rituximab
Safe, effective for newly diagnosed.
PEX required to achieve remission
Corticosteroids
Rapid immunosuppression
No firm evidence base to guide the choice of
steroid.
Favor : methylprednisolone IV 10 mg/kg/day.
Our standard : methylprednisolone 1 g iv/day
for 3 d (1st dose immediately administered
after 1st PEX)
Supportive Care
Antiplatelet
Platelet > 50 109/L.
Aspirin, Clopidogrel
Antithrombotic
TTP at hi risk of DVT. Administer DVT prophylaxis
Other Interventions
has such limited role e.g.:
Other immunosuppressant :mycophenolate mofetil,
cyclosporine, vincristine (yet no high quality data
regarding its efficacy)
Splenectomy : for relapsing disease (little good
evidence for its efficacy)
CONGENITAL TTP
Deleterious mutations within the ADAMTS13 gene.
Autoantibodies against ADAMTS13 do not occur in
Special Circumstances
Pregnancy
TTP may present during pregnancy
TTP in pregnancy may represent 1 of 2 processes:
acquired TTP due to the changes in immune regulation that
occur during pregnancy
precipitation of an episode of symptomatic TTP in a patient
with congenital TTP (which may or may not have been
previously recognized)
Patient treated w/ methylprednisolone and PEX in a
HIV-associated TTP
important cause of secondary TTP.
Patients w/ higher viral loads at presentation
Future directions
While the outcomes of patients have
Conclusions
Last decade : significant advances in
Thankyou