Thrombotic thrombocytopenic purpura laboratory findings

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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]

Overview

Laboratory Findings

  • CBC shows:
    • Thrombocytopenia (median platelet count 10,000/microL)
    • Microangiopathic hemolytic anemia [1]:
Hemolytic anemia
  • LDH ↑
  • Indirect bilirubin ↑
  • Serum haptoglubin ↓
  • Retic count ↑
  • Combs tests negative
  • Hb ~7 g/dl, Hct ~21%
  • Peripheral blood smear: Schistocytes, including helmet cells and triangular cells, polychromasia, microspherocytes and nucleated RBCs
  • Urinalysis: Hematuria, proteinuria
  • Serum creatinine: Increased
  • Urine output: Decreased
  • ADAMTS13 test: ADAMTS13 activity or inhibitor provides information for the diagnosis of the types and causes of TTP;
    • ADAMTS13 activity: Decreased to < 10% during acute episodes of TTP.
    • ADAMTS13 inhibitors test: This test is performed for patients with severe deficiency of ADAMTS13.
  • Genetic testing: Should be done in suspected cases of,
    • Positive family history
    • Recurrent episodes
    • Onset during childhood or pregnancy
    • Absence of inhibitors
    • Persistent ADAMTS13 deficiency
  • Imaging: In cases with higher suspicion of TTP, imaging is not necessary but with focal neurological signs MRI or CT may be considered
  • Blood culture: Patients with fever or signs and symptoms of infections
  • Stool exam: Stool culture and toxin evaluation should be considered in patients with diarrhea as one of the main presentation especially bloody diarrhea
  • Pathology: Tissue biopsy is not necessary for diagnosis, but it may show classic changes of a thrombotic microangiopathy including platelet microthrombi in small arterioles or capillaries, or hyaline changes in and around vessel walls.

References

  1. BRAIN MC, DACIE JV, HOURIHANE DO (1962). "Microangiopathic haemolytic anaemia: the possible role of vascular lesions in pathogenesis". Br J Haematol. 8: 358–74. PMID 14014893.

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