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aTTP is a Medical Emergency With Long Term Consequences

aTTP

Acquired

Thrombotic

Thrombocytopenic

Purpura

Acquired or immune-mediated thrombotic thrombocytopenic purpura (aTTP) is a rare blood disorder that develops when the immune system stops ADAMTS13 from working properly. This syndrome is characterized by anemia caused by damage to red blood cells accompanied by a low platelet count, neurologic symptoms, and renal disease.1

aTTP share many similarities with other blood diseases, whose treatment algorithms are different from aTTP. Thus, accurate diagnosis is an essential step to accurately prescribe an effective treatment. To do so, ADAMTS13 activity testing should be done.1
Your role is crucial to the diagnosis and speed of appropriate treatment for aTTP

ADAMTS13 Activity testing will help confirm or exclude your patient’s TTP disease diagnosis. In addition, the test results may assist clinical decision making in the treatment of aTTP.2

The management of exacerbations and disease relapse is an important aspect of treatment and follow-up of patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP), also known as acquired TTP. Severe ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency during clinical remission is associated with risk of relapse and may guide prophylactic immunemodulatory therapy.

Patients with TTP in remission should have regular ADAMTS13 activity measurements during follow- up. Although not based on a formal review of published evidence, it is suggested that ADAMTS13 levels should be assessed monthly for the first 3 months, every 3 months through the first year, and every 6–12 months thereafter.1 A decline in ADAMTS13 activity might warrant more frequent measurements, as persistently low levels may increase the risk for relapse.4,5

A blood sample for ADAMTS13 testing (ADAMTS13 activity and inhibitors or anti-ADAMTS13 IgG) should be obtained prior to initiation of therapeutic plasma exchange (TPE) or any blood product in a patient with clinical suspicion of TTP.4

References

  1. https://www.cablivi.com/attp/what-is-attp
  2. Zheng XL. ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura. Annu Rev Med. 2015;66:211–25
  3. Kremer Hovinga Strebel, J. A., & de la Rubia, J. (2022). The role of ADAMTS13 activity levels on disease exacerbation or relapse in patients with immune-mediated thrombotic thrombocytopenic purpura: Post hoc analysis of the phase 3 hercules and post-hercules studies. Blood, 140(Supplement 1), 5651–5653. https://doi.org/10.1182/blood-2022-156306
  4. Zheng XL, Vesely SK, Cataland SR, et al. Good practice statements (GPS) for the clinical care of patients with thrombotic thrombocytopenic purpura. J Thromb Haemost. Published online August 4, 2020. doi:10.1111/JTH.15009.
  5. Kremer Hovinga JA, Vesley SK, Terrell DR, Lämmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115(8):1500-1511.

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