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]

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

PLASMIC score

Platelet count (< 30,000/microL)
Hemolysis variables (Retic count > 2.5%, undetectable haptoglobin, indirect billi <2 mg/dL( >34 mcmol/L))
Absence of active cancer
Absence of transplant history (solid, stem cell)
MCV < 90 Fl
INR < 1.5
Serum creatinine < 2 mg/dL (<177 mcmol/L)

Score:

Risk of ADAMPTS13 deficiency in adults with suspected TTP :

• Low-risk = 0 to 4 points
• Intermediate risk = 5 points
• High-risk = 6 to 7 points