Incomplete thrombotic microangiopathy as a variant of hemolytic-uremic syndrome


Cite item

Full Text

Abstract

The described case illustrates difficulties in diagnosing atypical hemolytic-uremic syndrome (aHUS) in incomplete thrombotic microangiopathy (TMA) in the absence of thrombocytopenia, one of the signs of the classic triad of aHUS, which has resulted in the delayed verification of its diagnosis and in progressive kidney injury. The paper discusses the need to carry out kidney biopsy and to include sHUS in both the presence of a complete set of symptoms of this disease and in the absence of one of them into a range of diagnostic search.

References

  1. Ruggenenti P, Noris M, Remuzzi G. Trombotic mocroangiopathy, haemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Kidney Int. 2001;60:831-846.
  2. Noris M, Remuzzi G. Atypical haemolytic-uremic syndrome. N Engl J Med. 2009;361:1676-1687. doi: 10.1056/NEJMra0902814
  3. Kaplan BS, Meyers KE, Schulman SL. The pathogenesis and treatment of hemolytic uremic syndrome. J Am Soc Nephrol. 1998;9(6):1126-1133. doi: 10.5582/irdr.2014.01001.
  4. Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, Loirat C, Pecoraro C, Taylor CM, Van de Kar N, Vandewalle J, Zimmerhackl LB. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009;24:687-696. doi: 10.1007/s00467-008-0964-1.
  5. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher MA, Niaudet P, Guest G, Boudailliez B, Bouissou F, Deschenes G, Gie S, Tsimaratos M, Fischbach M, Morin D, Nivet H., Alberti C, Loirat C. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2007;18(8):2392-2400. doi: 10.1681/ASN.2006080811.
  6. Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide french series comparing children and adults. Clin JAm Soc Nephrol. 2013;8:554-562. doi: 10.2215/CJN.04760512.
  7. Veyradier A, Obert B, Houllier A, Meyer D, Girma JP. Specific von Willebrand factor — cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood. 2001;98(6):1765-1772. doi: 10.1182/blood.V98.6.1765.
  8. Fakhouri F, Roumenina L, Provot F, Sallee M, Caillard S, Couzi L, Essig M, Ribes D, Dragon-Durey MA, Bridoux F, Rondeau E, Frémeaux-Bacchi V. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21:859-867. doi: 10.1681/ASN.2009070706.
  9. De Serres SA, Isenring P. Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria. Nephrol Dial Transplant. 2009;24:1048-1050. doi: 10.1093/ndt/gfn687.
  10. Sallee M, Ismail K, Fakhouri F, Vacher-Coponat H, Moussi-Frances J, Fremaux-Bacchi V, Burtey S. Thrombocytopenia is not mandatory to diagnose haemolytic and uremic syndrome. BMC Nephrology. 2013;14(3):1471-2369. doi: 10.1186/1471-2369-14-3.
  11. Козловская Н.Л., Демьянова К.А., Кузнецов Д.В., Кучиева А.М., Боброва Л.А., Столяревич Е.С. «Субклиническая» тромботическая микроангиопатия при атипичном гемолитико-уремическом синдроме: единичный случай или закономерность? Нефрология и диализ. 2014;16(2):280-287.

Supplementary files

Supplementary Files
Action
1. JATS XML

Copyright (c) 2016 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
 

Address of the Editorial Office:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Correspondence address:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Managing Editor:

  • Tel.: +7 (926) 905-41-26
  • E-mail: e.gorbacheva@ter-arkhiv.ru

 

© 2018-2021 "Consilium Medicum" Publishing house


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies