Thrombotic thrombocytopenic purpura: Combined plasma exchange, fresh frozen plasma and corticosteroids

Background: Plasma exchange (PEX) plays an important role in the management of thrombotic thrombocytopenic purpura (TTP). Some studies had recommended the use of fresh frozen plasma (FFP) alone or with PEX. The use of corticosteroids was tried only in some studies with variable responses. This work was a retrospective study, carried out in the Plasma Exchange Unit, Zagazig University, Egypt, to study the outcome of the applied protocol of management of TTP, using combined therapeutic PEX, FFP infusion and pulse corticosteroids. Subjects and Methods: This study included 38 patients (28 females and 10 males) with TTP, mean age (37.1±12.7) years, referred to the Plasma Exchange Unit in the period from (2009-2013). They were divided into 2 groups as regards the therapy they received: Group I: 26 TTP patients (18 females and 8 males), who received daily plasma exchange sessions for 5 days, followed by every other day sessions, FFP infusion during and in-between sessions, in addition to methyl prednisolone pulse therapy followed by oral predinsolone, and Group II: 12 TTP patients (10 females and 2 males), who escaped one or more of the therapeutic protocol. Results: The overall survival among all the 38 TTP patients included 27 patients (71.1%); {19 females (67.9%) and 8 males (80%)}, in group I (26 patients), it included 24 patients (92.3%), {17 females (94.4%) and 7 males (87.5%)}, while in group II (12 patients), it included 3 patients (25%), {2 females (20%) and one male(50%)}. The overall number of plasma exchange sessions ranged from (1-14 sessions), with a mean of (7.1±3.5 sessions). Patients who died had sepsis, incomplete plasma removal, missed sessions, inadequate FFP infusion, delayed referral for plasma exchange, lack or delay of steroid use, complications related to TTP or plasma exchange itself or alternate diagnosis. Conclusion: The combined therapy for TTP, using a strict protocol of daily total volume PEX for 5 successive days, followed by every other day sessions, FFP infusion during and in-between sessions, in addition to methyl prednisolone pulse therapy, followed by oral prednisolone improves the outcome in TTP patients.