Early relapse of atypical hemolytic uremic syndrome following ABO-incompatible living-related pediatric kidney re-transplant successfully treated with eculizumab.
ABO-incompatible living
Eculizumab
Kidney transplant
Journal
Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728
Informations de publication
Date de publication:
10 2021
10 2021
Historique:
received:
05
04
2021
accepted:
08
06
2021
revised:
08
06
2021
pubmed:
18
7
2021
medline:
11
3
2022
entrez:
17
7
2021
Statut:
ppublish
Résumé
A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes. A 9-year-old girl received an ABO-incompatible (ABOi) living-related kidney transplant from her father with recipient and donor blood groups of O and A, respectively, with baseline recipient anti-A titers 1:128 reducing to 1:4 at the time of transplant with B lymphocyte depletion with rituximab and four sessions of immunoadsorption. Six hours post-transplant, she had recurrence of aHUS and received the first dose of eculizumab. She continues on monthly home eculizumab infusions with stable kidney allograft function and negative anti-A titers 7 years post-kidney transplantation. This is the first report of a pediatric high-risk ABOi living-related kidney transplantation in whom early relapse of aHUS was successfully treated with eculizumab with good long-term patient and allograft outcome.
Sections du résumé
BACKGROUND
A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes.
CASE-DIAGNOSIS/TREATMENT
A 9-year-old girl received an ABO-incompatible (ABOi) living-related kidney transplant from her father with recipient and donor blood groups of O and A, respectively, with baseline recipient anti-A titers 1:128 reducing to 1:4 at the time of transplant with B lymphocyte depletion with rituximab and four sessions of immunoadsorption. Six hours post-transplant, she had recurrence of aHUS and received the first dose of eculizumab. She continues on monthly home eculizumab infusions with stable kidney allograft function and negative anti-A titers 7 years post-kidney transplantation.
CONCLUSIONS
This is the first report of a pediatric high-risk ABOi living-related kidney transplantation in whom early relapse of aHUS was successfully treated with eculizumab with good long-term patient and allograft outcome.
Identifiants
pubmed: 34272986
doi: 10.1007/s00467-021-05193-7
pii: 10.1007/s00467-021-05193-7
doi:
Substances chimiques
Antibodies, Monoclonal, Humanized
0
eculizumab
A3ULP0F556
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3271-3275Subventions
Organisme : Department of Health
Pays : United Kingdom
Informations de copyright
© 2021. IPNA.
Références
Cochat P, Fargue S, Mastrallet G, Jungraithmayr T et al (2009) Disease recurrence in paediatric renal transplantation. Pediatr Nephrol 24:2097–2108
doi: 10.1007/s00467-009-1137-6
UK Renal Registry (2020) UK Renal Registry 22nd Annual Report - data to 31/12/2018, Bristol, UK. https://renal.org/audit-research/annual-report . The data reported here have been supplied by the UKRR of the Renal Association. The interpretation and reporting of these data are the responsibility of the authors and in no way should be seen as an official policy or interpretation of the UKRR or the Renal Association
Zuber J, Frimat M, Cailiard S, Kamar N, Gatault P (2019) Use of highly individualized complement blockage has revolutionized clinical outcomes after kidney transplantation and renal epidemiology of atypical hemolytic uremic syndrome. J Am Soc Nephrol 30:2449–2463
doi: 10.1681/ASN.2019040331
Kavanagh D, Goodship TH (2010) Atypical hemolytic uremic syndrome. Curr Opin Hematol 17:432–438
doi: 10.1097/MOH.0b013e32833cae86
Loirat C, Fremeaux-Bacchi V (2011) Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 6:60
doi: 10.1186/1750-1172-6-60
Nayer A, Asif A (2016) Atypical hemolytic-uremic syndrome: a clinical review. Am J Ther 23:151–158
doi: 10.1097/MJT.0b013e31829b59dc
Goodship J, Warwicker P, Pirson Y, Nichols A, Turnpenny P, Goodship T (1997) Hemolytic uremic syndrome maps to chromosome 1q and is associated with mutations in the complement factor H gene. Am J Hum Genet 61:A56
Taylor CM (2001) Hemolytic-uremic syndrome and complement factor H deficiency: clinical aspects. Semin Thromb Hemost 27:185–190
doi: 10.1055/s-2001-15247
Veyradier A, Obert B, Haddad E, Cloarec S, Nivet H et al (2003) Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome. J Pediatr 142:310–317
doi: 10.1067/mpd.2003.79
Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S et al (2013) Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8:554–562
Lemaire M, Fremeaux-Bacchi V, Schaefer F, Choi M, Tang WH, Le Quintrec M et al (2013) Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome. Nat Genet 45:531–536
doi: 10.1038/ng.2590
Loirat C, Fakhouri F, Ariceta G, Besbas N, BItzan M et al (2016) An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 31:15–39
doi: 10.1007/s00467-015-3076-8
Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N et al (2014) An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol 29:1967–1978
doi: 10.1007/s00467-014-2817-4
Gruppo RA, Rother RP (2009) Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med 360:544–546
doi: 10.1056/NEJMc0809959
Giordano M, Castellano G, Messina G, Divella C, Bellantuono R, Puteo F et al (2012) Preservation of renal function in atypical hemolytic uremic syndrome by eculizumab: a case report. Pediatrics 130:1385–1388
doi: 10.1542/peds.2011-1685
Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C et al (2013) Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 368:2169–2181
doi: 10.1056/NEJMoa1208981
Ahlenstiel T, Offner G, Strehlau J, Pape L, Froede K, Enrich JH, Schwartz A, Heuft HG, Klempner J (2006) ABO-incompatible kidney transplantation of an 8-yr-old girl with donor/recipient-constellation A1B/B. Xenotransplantation 13:141–147
doi: 10.1111/j.1399-3089.2006.00279.x
Ohta T, Kawaguchi H, Hattori M, Takahashi K et al (2000) ABO-incompatible pediatric kidney transplantation in a single-centre trial. Pediatr Nephrol 14:1–5
pubmed: 10654320
Mamode N, Marks SD (2013) Maximising living donation with paediatric blood-group-incompatible renal transplantation. Pediatr Nephrol 28:1037–1040
doi: 10.1007/s00467-012-2279-5
Stojanovic J, Adamusiak A, Kessaris N, Chandak P, Ahmed Z, Sebire NJ, Walsh G, Jones HE, Marks SD, Mamode N (2017) Immune desensitization allows pediatric blood group incompatible kidney transplantation. Transplantation 101:1242–1246
doi: 10.1097/TP.0000000000001325
Kulasekararaj AG, Hill A, Rottinghaus ST, Langermeijer S et al (2019) Ravulizumab (ALEX1210) vs eculizumab in C5-inhibitor-experienced adult patients with PHN: the 302 study. Blood 133:540–549
doi: 10.1182/blood-2018-09-876805
Rees L, Kim JJ (2015) HLA sensitisation: can it be prevented? Pediatr Nephrol 30:577–587
doi: 10.1007/s00467-014-2868-6
McKeage K (2019) Ravulizumab: first global approval. Drugs 79:347–352
doi: 10.1007/s40265-019-01068-2