Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange

Abstract

The aim of this study was to investigate pretreatment prognostic factors that could be useful in predicting the response to plasma exchange in thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). Thirty-two patients with TTP/HUS, treated with plasma exchange at our institution from 1980 to 1994, were studied. The main clinical and laboratory data at the beginning of plasma exchanges were analyzed by the Cox stepwise logistic regression, applied to either treatment failure or death. Seventeen (53%) patients attained a complete remission and 22 (69%) survived (five in advanced renal failure and long-term hemodialysis). Longer delay in initiating plasma exchanges, presence of stupor or coma, and higher creatinine levels at the beginning of plasma exchanges were independent predictors of treatment failure. Stupor or coma at the beginning of plasma exchanges was the only predictor of mortality from unremitted TTP/HUS. Hemoglobin levels, platelet count, and LDH activity, traditionally envisaged as markers of disease activity, neither correlated with previous duration of TTP/HUS nor had any prognostic value. Early diagnosis of TTP/HUS and prompt initiation of intensive plasma exchange emerged from this study as the most effective interventions for improving the prognosis of TTP/HUS patients.

Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment

“…The excellent response rate of relapsed TTP probably relates to the milder clinical presentation and the shorter delay in initiating PE. Long delays in making the diagnosis of TTP and initiating PE have been associated with progression of CNS involvement and treatment failure [5,12].…”

Management of thrombotic thrombocytopenic purpura: current perspectives

“…Delay in initiation of PEX is a major factor in adverse outcomes12 and it is therefore our practice to initiate PEX in patients with TTP transferred to our institution regardless of time of day. PEX is associated with a reduced death rate and is superior to plasma infusion alone, which is the result of the additive effects of both removal of autoantibody by the exchange process (in the case of acquired TTP) as well as supplementing ADAMTS13 activity in the exchanged plasma 13.…”

A systematic review of randomized controlled trials for plasma exchange in the treatment of thrombotic thrombocytopenic purpura

“…Thrombotic thrombocytopenic purpura (TTP) is a rare, often fatal blood disorder characterized by thrombocytopenia and microangiopathic haemolytic anaemia (Allford et al , 2003). Early instigation of treatment following a diagnosis of TTP is vital, as delays have been shown to adversely affect outcome (Pereira et al , 1995). The mainstay of treatment is therapeutic plasma exchange (PE), both to remove the causative antibody to von Willebrand factor cleaving metalloprotase (ADAMTS13) and to replace ADAMST13 (Furlan et al , 1997; Rock, 2005).…”