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Distinguishing features between VITT, ITP, and TTP

Distinguishing features between VITT, ITP, and TTP
  Syndrome
VITT or VITT-like disorder ITP TTP
Thrombocytopenia
  • Yes, typically 10,000 to 100,000/microL
  • Yes
  • Yes
Thrombosis
  • Yes, including atypical sites of venous and arterial thrombosis
  • Generally not seen, although there may be a slightly increased risk for VTE
  • Typically microvascular rather than VTE
Other clinical
  • Adenovirus infection or other trigger (eg, monoclonal gammopathy)
  • Flu-like syndrome
  • Petechiae or purpura
  • Often, otherwise well; often an incidental finding
  • Neurologic, kidney, and/or cardiac involvement may be seen
Other laboratory
  • Normal to slightly prolonged PT and aPTT
  • Fibrinogen may be low
  • D-dimer often markedly increased
  • Normal PT and aPTT
  • Normal fibrinogen and D-dimer
  • Normal hemoglobin (unless anemia from bleeding)
  • Normal PT and aPTT
  • Normal fibrinogen and D-dimer
  • Microangiopathic hemolytic anemia with laboratory findings of hemolysis and schistocytes on the blood smear
Diagnostic confirmation
  • Positive anti-PF4 antibody ELISA or functional assay
  • Diagnosis of exclusion
  • Severe ADAMTS13 deficiency (activity <10%)
Management implications*
  • Anticoagulation
  • Avoid warfarin (unless platelet count has recovered)
  • High-dose IVIG
  • Minimize platelet and plasma transfusions
  • Platelet transfusions for critical bleeding
  • Glucocorticoids or IVIG for serious bleeding or severe thrombocytopenia
  • Rituximab, splenectomy, TPO-RA, or fostamatinib in selected cases
  • Therapeutic plasma exchange
  • Glucocorticoids
  • Rituximab
  • Caplacizumab in selected cases
  • Avoid platelet transfusions unless major bleeding
Bolded text identifies some of the key distinguishing features of VITT. Hematologist input is advised to assist in distinguishing among these and other syndromes with thrombocytopenia and thrombosis.

aPTT: activated partial thromboplastin time; COVID-19: coronavirus disease 2019; ELISA: enzyme-linked immunosorbent assay; ITP: immune thrombocytopenia; IVIG: intravenous immune globulin; PT: prothrombin time; TPO-RA: thrombopoietin receptor agonist; TTP: thrombotic thrombocytopenia; VITT: virus-induced immune thrombotic thrombocytopenia, previously called vaccine-induced immune thrombotic thrombocytopenia; VTE: venous thromboembolism.

* This is only intended to summarize major differences in management and to emphasize the importance of making the correct diagnosis. Refer to UpToDate for detailed management discussions.

Graphic 131494 Version 2.0

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