Efficacy of rituximab and plasmapharesis in an adult patient with antifactor H autoantibody-associated hemolytic uremic syndrome: A case report and literature review.
Antifactor H antibody (anti-CFHAb) is found in 6% to 25% cases of atypical hemolytic uremic syndrome (aHUS) in children, but has been only exceptionally reported in adults. There is no consensus about the best treatment for this type of aHUS. We report the case of an adult patient treated successfully with plasma exchange (PE), steroids, and rituximab.A 27-year-old Caucasian male presented to hospital with anemia, thrombocytopenia, and acute renal failure. One week earlier, he had digestive problems with diarrhea. The diagnosis of anti-CFHAb-associated aHUS (82,000 AU/mL) without CFHR gene mutations was established.He received Rituximab 375 mg/m (4 pulses) with PE and steroids. This treatment achieved renal and hematological remission at day (D) 31 and negative anti-CFHAb at D45 (<100 AU/mL). At D76, a fifth rituximab pulse was performed while CD19 was higher than 10/mm. Steroids were stopped at month (M) 9. The patient has not relapsed during long-term follow-up (M39).Rituximab therapy can be considered for anti-CFHAb-associated aHUS. Monitoring of anti-CFHAb titer may help to guide maintenance therapeutic strategies including Rituximab infusion.
aCHU Clermont-Ferrand, Service de Néphrologie, Pôle REUNNIRH, Clermont-Ferrand bCHU Paris Est, Hôpital Saint-Antoine, Service d'Hématologie, AP-HP cCentre de Référence des Microangiopathies Thrombotiques dUniversité Pierre et Marie Curie UPMC Université Paris 6, Paris eCHU Clermont-Ferrand, Service d'Immunologie fCHU Clermont-Ferrand, Service de Réanimation, Pôle REUNNIRH, Clermont-Ferrand, France.
Clemence
C
Deville
Cyril
C
Garrouste
Paul
P
Coppo
Bertrand
B
Evrard
Alexandre
A
Lautrette
Anne Elisabeth
AE
Heng
D000328:Adult; D065766:Atypical Hemolytic Uremic Syndrome / Q000097:blood / Q000276:immunology / Q000628:therapy*; D001323:Autoantibodies / Q000097:blood*; D017242:Complement Factor H / Q000276:immunology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D010956:Plasmapheresis / Q000379:methods*; D000069283:Rituximab / Q000627:therapeutic use*
D001323:Autoantibodies; D007155:Immunologic Factors; D000069283:Rituximab; D017242:Complement Factor H
Nefrologia. 2013 Jan 18;33(1):27-45
23364625
Br J Haematol. 2014 Mar;164(6):759-66
24387053
Mol Immunol. 2013 Dec 15;56(3):170-80
23830046
Am J Transplant. 2009 May;9(5):1223-9
19422347
Blood. 2004 Nov 1;104(9):2635-42
15226177
Clin Exp Nephrol. 2016 Apr;20(2):265-72
26156042
Pediatr Nephrol. 2015 Mar;30(3):451-7
25217328
Kidney Int. 2014 May;85(5):1019-22
24786877
J Am Soc Nephrol. 2010 Dec;21(12):2180-7
21051740
Am J Kidney Dis. 2014 Jan;63(1):40-8
24021908
Nephrol Dial Transplant. 2011 Nov;26(11):3811-3
21813829
J Am Soc Nephrol. 2005 Feb;16(2):555-63
15590760
Nat Rev Nephrol. 2013 Mar;9(3):154-69
23338210
NDT Plus. 2009 Dec;2(6):458-60
25984036
Orphanet J Rare Dis. 2011 Sep 08;6:60
21902819
Blood. 2007 Sep 1;110(5):1516-8
17495132
Pediatr Nephrol. 2014 Jan;29(1):75-83
23868108
Kidney Int. 2014 May;85(5):1151-60
24088957
Clin J Am Soc Nephrol. 2013 Mar;8(3):407-15
23243267
Eur J Intern Med. 2015 Nov;26(9):659-65
26293834
N Engl J Med. 2014 Aug 14;371(7):654-66
25119611
Pediatr Nephrol. 2016 Jan;31(1):15-39
25859752
Clin J Am Soc Nephrol. 2007 May;2(3):591-6
17699467
Clin J Am Soc Nephrol. 2013 Apr;8(4):554-62
23307876