Thrombotic thrombocytopenic purpura diagnostic study of choice
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Saeedeh Kowsarnia M.D.[2],Sogand Goudarzi, MD [3]
Overview
Study of choice
- ADAMTS13 test:
Studies that may be considered for diagnosis of the suspected cases of TTP:
- Complete blood count (CBC) with platelet count
- Review of the peripheral blood smear
- Serum electrolytes and creatinine
- Serum lactate dehydrogenase (LDH)
- Serum bilirubin level
- Coagulation testing ( PT, aPTT, fibrinogen, D-dimer)
- Serum haptoglobin level
- Direct antiglobulin (Coombs) test (DAT)
- ADAMTS13 activity and inhibitor testing
- Stool exam
- Genetic testing for mutation in ADAMTS13 at the genomic level
- Antibodies to complement factors H and I for ruling out atypical hemolytic uremic syndrome
PLASMIC score
Risk of ADAMTS13 deficiency in adults with suspected TTP :
- Low-risk = 0 to 4 points
- Intermediate risk = 5 points
- High-risk = 6 to 7 points