Key Takeaways

✅ Recent onset of thrombocytopenia and hemolytic anemia is consistent with a diagnosis of Evans syndrome, thrombotic microangiopathies (such as thrombotic thrombocytopenic purpura [TTP], hemolytic uremic syndrome [HUS] or disseminated intravascular coagulation [DIC]), infection or primary bone marrow process such as acute leukemia.

✅ TTP is distinguished from other types of thrombotic microangiopathies by virtue of normal (or near normal) renal function and coagulation assays.

✅ Diagnosis of TTP is confirmed by ADAMTS13 activity levels, but treatment decisions must be made while this test is pending.

✅ First-line therapy for patients who have a high probability of TTP (based on clinical assessment +/- clinical prediction score) consists of plasma exchange and steroids.

✅ Other therapies may be considered, including rituximab and caplacizumab, but these are added to plasma exchange/steroids, rather than replacing them.