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Membrane‐filtration based plasma exchanges for atypical hemolytic uremic syndrome: Audit of efficacy and safety
Author(s) -
Khandelwal Priyanka,
Thomas Christy C.,
Rathi Bhim Singh,
Hari Pankaj,
Tiwari Anand N.,
Sinha Aditi,
Bagga Arvind
Publication year - 2019
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.21711
Subject(s) - medicine , atypical hemolytic uremic syndrome , eculizumab , adverse effect , chills , gastroenterology , renal function , dialysis , surgery , antibody , complement system , immunology
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 m 2 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.