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

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 ADAMTS13 deficiency in adults with suspected TTP :

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

References