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. 2019 Oct;34(5):555-562.
doi: 10.1002/jca.21711. Epub 2019 Jun 7.

Membrane-filtration based plasma exchanges for atypical hemolytic uremic syndrome: Audit of efficacy and safety

Priyanka Khandelwal  1 , Christy C Thomas  1 , Bhim Singh Rathi  1 , Pankaj Hari  1 , Anand N Tiwari  1 , Aditi Sinha  1 , Arvind Bagga  1
Affiliations

Membrane-filtration based plasma exchanges for atypical hemolytic uremic syndrome: Audit of efficacy and safety

Priyanka Khandelwal et al. J Clin Apher. 2019 Oct.

Abstract

Background: While complement blockade with eculizumab is recommended as first-line therapy of atypical hemolytic uremic syndrome (aHUS), plasma exchanges (PEX) remain the chief option for anti-factor H (FH) antibody associated disease and when access to eculizumab is limited.

Methods: We reviewed adverse events (AEs) and adverse outcomes (eGFR <30 mL/min/1.73 m2 or death), in all patients with aHUS managed with membrane-filtration based PEX at one tertiary care center over 5.5 years.

Results: During January 2013 to June 2018, 109 patients with aHUS (74 with antibodies to FH), aged median (range) 7.6 (0.5-18) year weighing 22.1 (6-90) kg, underwent 2024 sessions of PEX. AE, in 12.1% patients, were usually self-limiting and included chills (5.5%), vomiting/abdominal pain (3.3%), hypotension (1.6%), urticaria (1.5%), seizures (0.2%), hypocalcemia (0.2%), and hemorrhage (0.1%); plasma hypersensitivity and severe reactions were rare. Rate of catheter-related infections was 1.45/1000 catheter-days. Filter reuse (OR 1.69; 95% CI 1.26-2.26; P < .001) and >20 sessions of PEX/patient (OR 1.99; 95% CI 1.27-3.10; P = .002) were independently associated with adverse events; infusion of IV calcium gluconate during PEX was protective (OR 0.26; 95% CI 0.16-0.43; P < .001). Hematological remission was achieved in 96.3% patients after 6 (5-8) PEX sessions; 80.8% and 89.6% patients were dialysis independent by one and 3 months, respectively.

Conclusions: PEX is safe and associated with satisfactory short-term outcomes in children with aHUS. Prolonged PEX and filter-reuse are associated with complications.

Keywords: central venous catheter; clinical audit; complement factor H; plasmapheresis.

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References

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