New Concepts in the Diagnosis and Management of Thrombotic Thrombocytopenic Purpura, Hemolytic Uremic Syndrome and Related Disorders

Dear Colleagues,

For many years, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) were viewed as clinical syndromes that shared features of microangiopathic anemia (MAHA) and thrombocytopenia—HUS for patients with prominent renal failure and TTP for those without it. These syndrome-based definitions of TTP and HUS were problematic, as there was no biologic basis to determine the threshold of renal failure for the diagnosis of HUS. Furthermore, some patients with relapses were found to meet the working criteria of TTP on some occasions and those of HUS on others. To resolve this dilemma, the hybrid term of TTP/HUS was invented to encompass both syndromes, at least in adult practices. In accordance with this hybrid diagnosis, most clinicians treated TTP and HUS as one and the same. Advances at the end of the last century in identifying the molecular pathogenesis of TTP and HUS led to paradigm shifts in our concepts of TTP, HUS, and other disorders presenting with MAHA and thrombocytopenia. The journal Medicina is launching this Special Issue to provide a state-of-the-art overview of this subject. We invite authors to submit reports of original research or review articles dealing with disease classification, pathogenesis, pathophysiology, diagnosis, and management. Case reports that provide novel insights are also welcome.

Dr. Han Mou Tsai
Guest Editor

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• thrombotic thrombocytopenic purpura
• atypical hemolytic uremic syndrome
• typical hemolytic uremic syndrome
• ADAMTS13
• complement activation
• von Willebrand factor
• shear stress
• shiga toxins
• thrombotic microangiopathy
• experimental models