High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome. Coppo P, Bussel A, Charrier S, Adrie C, Galicier L, Boulanger E, Veyradier A, Leblanc T, Alberti C, Azoulay E, Le Gall JR, Schlemmer B. Medicine (Baltimore) 2003;82(1):27-38.

Thrombotic thrombocytopenic purpura (TTP) and adult hemolytic-uremic syndrome (HUS) have a substantial mortality rate even with currently available treatments. Although therapeutic plasma exchange (TPE) is the recommended treatment of TTP/HUS, this cumbersome procedure may not be available for all patients in an emergency. In this context, plasma infusion may represent an alternative first-line therapy. The authors compared the effectiveness of high-dose plasma infusion (HD-PI; 25-30 mL/kg per day) and TPE as first-line treatment of adult TTP/HUS at a single hospital in Paris, France between 1989 and 2001. Patients whose outcome directly depended on underlying diseases with poor prognosis and which are usually nonresponsive to conventional treatment were excluded. Two groups of patients with TTP/HUS were identified according to their initial therapy, that is, HD-PI (19 patients) and TPE (18 patients). Patients of the two groups had comparable clinical and laboratory features on admission. Associated medical conditions were identified in 19 patients, including 14 patients with documented infections. Sixteen patients achieved complete remission (total reversal of clinical manifestations and thrombocytopenia) in each group. Median times to recovery of platelet counts and LDH levels were comparable between the two groups. Eight patients in the HD-PI group were switched to TPE because of fluid overload (6 patients), persistent biologic disturbances (1 patient), or unresponsiveness to HD-PI treatment (1 patient). This latter patient had severe TTP/HUS that remained refractory to TPE and vincristine and rapidly died. All 7 remaining patients achieved complete remission with TPE. Four patients in the HD-PI group died; two deaths were caused by multiorgan failure related to TTP/HUS, one death occurred in a patient refractory to treatment who died of a brain hemorrhage, and one patient died of a gastrointestinal hemorrhage after remission. Three patients in the TPE group died; two deaths were caused by multiorgan failure related to refractory TTP/HUS and one patient experienced a fatal exacerbation of the disease five days after complete remission. In the HD-PI group, 5 patients experienced a transient nephrotic-range proteinuria during treatment. Main complications in the TPE group were collapse (1 patient) and central venous catheter infection (2 patients) and thrombosis (1 patient). Three patients in each group relapsed (defined as the reappearance of clinical disturbances and/or thrombocytopenia after 7 consecutive days of complete remission). HD-PI may be an efficient treatment of TTP/HUS in patients who cannot have early plasma exchange. However, the large volumes of plasma required to reach complete remission may result in fluid overload, which may necessitate subsequent TPE.