Progress in Hematology Recent advance in thrombotic thrombocytopenic purpura

International Journal of Hematology

, Volume 91, Issue 1, pp 36-45

First online:

Thrombotic thrombocytopenic purpura: recognition and management

  • Joseph E. KissAffiliated withDivision of Hematology/Oncology, Department of Medicine, University of Pittsburgh School of MedicineTherapeutic Hemapheresis and Blood Services, The Institute for Transfusion Medicine Email author 

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Thrombotic thrombocytopenic purpura is a life-threatening multisystem disorder that represents both a diagnostic and a management challenge to clinicians. Early recognition of the condition coupled with rapid institution of plasma exchange has led to a dramatic improvement in prognosis. Studies performed over the past decade have elucidated the predominant pathophysiology, stemming from a deficiency of ADAMTS13, that accounts for the widespread microvascular deposition of platelet–von Willebrand factor in many sites, including the brain, kidney, and mesenteric vessels. However, in light of the mortality rate of 10–20%, much work remains to be done to translate advances in our understanding of pathophysiology into clinical practice. Improvements in medical management using immunosuppressive and other drugs are being actively explored in clinical trials. Agents that target ADAMTS13 autoantibody production by B-cells, such as anti-CD20 monoclonal antibodies, have the potential to shorten the duration of plasma exchange treatment, reduce relapses, and transform the management of this once enigmatic disorder.


Thrombotic thrombocytopenic purpura (TTP) Thrombotic microangiopathy Plasma exchange ADAMTS13