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Case 87
Published in Atul B. Mehta, Keith Gomez, Clinical Haematology, 2017
The cornerstone of treatment is plasma exchange. Solvent detergent plasma is preferred to reduce the risk of transmission of transfusion associated infections and allergic reactions, but if not available then fresh-frozen plasma (FFP) or cryosupernatant may be used. Daily plasma exchanges should be continued until a minimum of 2 days after normalisation of the platelet count.
Management of immune thrombotic thrombocytopenic purpura with caplacizumab: a Canadian, single-centre, real-world experience
Published in Platelets, 2023
Brandon Tse, Megan Buchholz, Katerina Pavenski
Treatment details for each patient are described in Table II. All patients received TPE within 24 h of admission. The median number of exchanges was 9 (IQR 7.0–12.5). With regard to replacement fluid, five patients received only cryosupernatant plasma (CSP), two patients received only solvent detergent plasma (SDP), and four patients received both CSP and SDP during treatment. All patients received high-dose of steroids of 1 mg/kg/day (or higher) daily until clinical remission, followed by a taper of 10 mg per week. Eight patients (73%) received at least one dose of rituximab or biosimilar during admission, which was mostly started in the setting of refractory disease (started median of 7.5 days into admission, IQR 6.8–12.5 days). Rituximab was administered in the same dose and frequency (375 mg/m2 weekly for 4 weeks) unless otherwise noted.
Thrombotic thrombocytopenic purpura in a 2.5-year-old boy with dengue infection: a rare complication
Published in Paediatrics and International Child Health, 2020
Rajasekhar Reddy Gogireddy, Vasanth Kumar, Suchitra Ranjit, Rajeswari Natraj, Priyavarthini Venkatachalapathy, Indira Jayakumar, Saravanan Margabandhu
In view of the possibility of dengue-associated thrombotic microangiopathy (TMA), urgent plasma exchange and renal replacement therapy in the form of sustained low efficiency dialysis (SLED) was initiated. Initially, double volume plasma exchange was undertaken daily with cryosupernatant plasma. Unfortunately, the ADAMTS13 activity assay sent before the plasma exchange commenced was clotted. Repeat samples sent after three plasma exchanges showed normal activity. Plasma exchange was continued once daily for 3 days and then on alternate days. He continued to remain anuric, hypertensive and had fluid overload, requiring daily SLED for 11 days and multiple anti-hypertensive drugs. On Day 8 of hospital stay and after four cycles of plasma exchange, his general condition began to improve. The platelet count subsequently improved slowly to normal (400 × 109/L) and the serum LDH level showed a steady decline to 9.4 µkat/L (Figure 1). He underwent seven cycles of plasma exchange over 11 days. On Day 10 of hospitalisation, he was successfully extubated and was conscious and oriented with no sensory motor deficit. He was followed up for 6 months and remains well with a normal haemogram.
Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura
Published in Expert Review of Hematology, 2019
To date, exogenous plasma is the only way to bring ADAMTS13 to the deficient, iTTP patient. Thus, there is a need to optimize the treatment using plasma as the sole replacement fluid for TPE. Several preparations of human plasma have been used, differing in the use and nature of a technology for pathogen reduction or inactivation. The physical or chemical processes to which plasma is subjected for safety are likely to affect their qualitative content, especially regarding proteins involved in hemostasis. Indeed, ADAMTS13 activity seems to be preserved in all kinds of plasma preparation [51,52]. In contrast, solvent detergent plasma appears to have a reduced content in high-molecular-weight vWF [52]. Several studies, mostly retrospective and small sized, have looked for clinical consequences of these disparities when each plasma was used as replacement fluid for iTTP. Overall, no differences in efficacy could be demonstrated between the historical quarantined fresh frozen plasma, solvent–detergent plasma, or amotosalen-inactivated plasma [53–55]. However, the use of solvent–detergent or amotosalen-inactivated plasmas could be associated with a lower incidence of plasma-related adverse events [54,56]. Additionally, while remission rates did not differ, a faster platelet count recovery has been observed with the use of amotosalen-inactivated plasma [55] [Garraud et al., in preparation]. The use of methylene-blue inactivated plasma has been discontinued in several countries due to allergy concerns. Moreover, such plasma seemed to be less effective when compared with fresh frozen plasma [57–59]. Finally, independently of the safety measure, a cryoprecipitate-poor plasma can be obtained after slow thawing of a frozen plasma and removal of the formed sediment. The theoretical interest of this preparation, variously referred as cryoprecipitate-poor, cryosupernatant, or cryodepleted plasma, lies in its depletion in ‘cryofactors’, including vWF [52]. Nevertheless, cryosupernatant plasma did not show clear superiority in any of the three trials that evaluated its use as a replacement fluid for TPE in iTTP [60–62]. Moreover, such preparation is not routinely available in all countries.