When to suspect TTP?
TTP is rare and presents similarly to other thrombotic microangiopathies (TMAs), making it difficult to diagnose.
aTTP is a medical emergency: diagnosing aTTP rapidly is critical and can save a life1
When a patient presents with:1
Severe thrombocytopenia
(often platelets <30 × 109/L)
Microangiopathic hemolytic anemia (MAHA)
characterized by the presence of schistocytes in blood smear
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With no identified cause
Why TTP can be difficult to diagnose?
TTP is rare and presents similarly to other thrombotic microangiopathies (TMAs), making it a challenge to diagnose. All TMAs result in thrombosis of capillaries and arterioles due to endothelial injury.2,3
*STEC-HUS – Hemolytic Uremic Syndrome mediated by E. coli producing Shiga-type toxin or verotoxin;
HUS: Hemolytic Uremic Syndrome
Adapted from Kremer Hovinga et al. Nat Rev Dis Primers 20176
Tests and expected results in patients with suspected aTTP1
Diagnosis of TTP
Dr Marie Scully shares details on how to make a prompt differential diagnosis of TTP.
Références
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Scully M, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323–35.
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Arnold DM, Patriquin CJ, Nazy I. Thrombotic microangiopathies: a general approach to diagnosis and management. CMAJ. 2017;189(4):E153-E159. doi:10.1503/cmaj.160142
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Tsai H-M. Pathophysiology of thrombotic thrombocytopenic purpura. Int J Hematol. 2010;91(1):1-19. doi:10.1007/s12185-009-0476-1
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Tsai H-M, et al. Antibodies to von Willebrand Factor-cleaving protease in acute thrombotic thrombocytopenic purpura.N Engl J Med. 1998;339:1585–94.
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Furlan M, et al. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89: 3097–103.
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Kremer Hovinga JA, et al. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017;3:17020.
