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.