Thrombotic Thrombocytopenic Purpura (TTP)
- Microangiopathic Hemolytic Anemia (“MAHA,” with schistocytes in the peripheral smear and lab evidence of intravascular hemolysis)
- Renal failure
- Mental status changes
However, according to the 2013 and 2016 American Society for Apheresis Guidelines, you don’t need to wait for all five, as “…the clinical findings of unexplained thrombocytopenia and MAHA are sufficient to diagnose TTP” (Schwartz J et al, “Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue” J Clin Apher 2013;28:145–284). This means that it is not only a mistake to wait until all five parts of the “pentad” are present to initiate therapy, but that waiting the often one week or so turnaround time for ADAMTS13 levels to confirm the diagnosis is another very big mistake. Therapeutic plasma exchange (with plasma replacement rather than albumin) is the treatment of choice, and should be initiated early and continued daily until the platelet count rises to above 150,000/mcL.
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