TTP/HUS and prognosis: the syndrome and the disease(s).
Patients presenting with the syndrome of microangiopathic hemolysis and thrombocytopenia, without Shiga toxin-associated colitis, have thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). Prognosis for TTP/HUS has changed over time from a fatal disorder associated with the classic pentad to a syndrome associated with 80% survival in the plasma exchange era. A growing number of mechanisms, including but not exclusive to severe ADAMTS13 deficiency, are now understood to result in this syndrome, and the prognosis of patients with TTP/HUS is related to many additional factors. This update on prognosis explores recent registry data studying both acquired idiopathic and also familial or recurrent forms of TTP/HUS, to delineate how mortality varies by underlying disease mechanism. This paper also explores the association between mortality and clinical presenting features, as well as whether the case is a primary or relapsed presentation. Recent data support an understanding of TTP/HUS as a heterogeneous syndrome with variable mortality, and with specific subgroups demonstrating an excellent outcome.
Forzley
Brian R
BR
Department of Medicine, University of Calgary, Calgary, Alberta, Canada. brian.forzley@calgaryhealthregion.ca
Clark
William F
WF
D015982:Bias; D015986:Confounding Factors, Epidemiologic; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D006801:Humans; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D012042:Registries; D012307:Risk Factors; D009626:Terminology as Topic; D016896:Treatment Outcome