Thrombotic Microangiophatic in the ICU, postpartum hemolytic uremic syndrome: case report
PDF (Español)


Hemolytic Uremic Syndrome atypical
thrombotic microangiopathy
pregnancy. (MeSHsource)

How to Cite

Urbina Z, Soledad R, Córdoba JP. Thrombotic Microangiophatic in the ICU, postpartum hemolytic uremic syndrome: case report. Rev. Colomb. Nefrol. [Internet]. 2016 Jul. 11 [cited 2024 Apr. 20];3(1):41-5. Available from:


Underlying injures produced by Hemolytic Uremic Syndrome, a clinical entity defined by the triad, inclu-de:  non-immune hemolytic anemia, thrombocytopenia, and organ involvement which are measured by the Trombotic Systemic Microangiopathy (TSM)  process. The atypical HUS (HUSa) is a subtype of HUS in which the phenomena of TSM are the result of the lack of regulation in alternative pathway of complements on cell surfaces, secondary to a genetic cause. The key role played by the deregulation of the complement system on the damaged endothelial layer in patients with HUSa has been established;   this system measures by multiple mutations and polymorphisms in the genes that encode certain regulatory proteins of the add-in has been established. Taking into account the great complement activity of physiological way during ges-tation, each day more cases of HUSa related to pregnancy, are described.  There is a monoclonal antibody, Eculizumab, which inhibits the terminal complement fraction (C5). Eculizumab blocks the formation of the attack complex of the membrane, with significant long-term improvement in morbidity and mortality asso-ciated with this disease, promoting long-term recovery of renal function and with a significant reduction in the need for dialysis or plasma therapy.A case of 17 year in puerperium, who developed icteric syndrome considered as HELLP syndrome, whose atypical evolution made us think about alternative diagnosis of thrombotic microangiopathy in pregnant woman HUSa type. Discard diagnosis between sepsis, HELLP syndrome and disseminated intravascular coagulation was made.  ADAMST13 measurement was taken, whose standard value allowed us to make the differential diagnosis with a Thrombotic Thrombocytopenic Purpura (TTP). In this way, we were able to achieve the clinical diagnosis of HUSa and begin treatment.
PDF (Español)


1. Mok C, Raymond C, Kwok L, Paul SF. Effect of renal disease on the standardized mortality ratio and life expectancy of patients with systemic lupus erythematosus. Arthritis and Rheumatism. 2013;65:2154–60.

2. Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L,et al.Pregnancy-Associated Hemolytic Uremic SyndromeRevisited in the Era of Complement Gene Mutations. J Am SocNephrol.2010;21:859–867. doi: 10.1681/ASN.2009070706.

3. Noris M, Caprioli J, Bresina E, Mossali C, Pianetti G, et al. Relative Role of genetic Complement abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype. Clin J Am SocNephrol. 2010;5(10):1844–59.

4. Córdoba JP, Contreras KM, Larrarte C, Espitaleta Z, González LE, Ibarra M. et al. Síndrome hemolítico urémico atípico, revisión de la literatura y documento de consenso.Revista Colombiana de Nefrologia. 2015;2(1):19–40.

5. Siegler R, Oakes R. Hemolytic uremic syndrome; pathogenesis, treatment, and outcome.CurrOpinPediatr. 2005;17(2):200–4.

6. Campistol JM, Arias M, Ariceta G, Blasco M, Espinosa M, Grinyó JM, et al. Actualización en síndrome heolítico urémico atípico: diagnóstico y tratamiento.Nefrología.2013;33(1):27–45.

7. Loirat C, Saland J, Bitzan M. Management of hemolytic uremic syndrome. Presse Med. 2012 Mar;41(3 Pt 2):e115-35. doi: 10.1016/j.lpm.2011.11.013. Epub 2012 Jan 27.

8. ConstantinescuAR, Bitzan M, Weiss LS, Christen E, Kaplan BS, Cnaan A, et al. Non-enteropathic hemolytic uremic syndrome: causes and short-term course.Am J Kidney Dis. 2004 Jun;43(6):976–82.

9. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA. French Society of Pediatric Nephrology. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome.J Am Soc Nephrol.2007;18:2392–2400. doi: 10.1681/ASN.2006080811.

10. Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome.Orphanet Journal of Rare Diseases. 2011;6:60.doi:10.1186/1750-1172-6-60.

11. Caprioli J, Noris M,Brioschi S, Pianetti G,Castelletti F, Bettinaglio P. Genetics of HUS:the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, andoutcome. Blood. 2006 Aug 15;108(4):1267–79. Epub 2006 Apr 18.

12. Noris M, Caprioli J, Bresina E, Mossali C, Pianetti G. Relative Role of genetic Complement abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype.Clin J Am Soc Nephrol. 2010 Oct;5(10):1844–59. doi:10.2215/CJN.02210310. Epub 2010 Jul 1.

13. Neuhaus TJ, Calonder S, Leumann EP. Heterogeneity of atypical haemolytic uraemic syndromes. Arch Dis Child. 1997 Jun;76(6):518–21.

14. Ohanian M, Cable C,Halka K. Eculizumad safely reverses neurologic impairment and eliminates need for dialysis in sevee atypical hemolytic uremic syndrome.Clinical Pharmacology: Advances and Applications. 2011;3:5–12.

15. Cofiel R, Kukreja A,Bedard K, Yan Y, Mickle A,Ogawa M, et al. Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS. Blood. 2015 May 21;125(21):3253–62. doi: 10.1182/blood-2014-09-600411. Epub 2015 Apr 1.

16. Nester CM. Managing atypical hemolytic uremic syndrome: chapter 2.Kidney nternational. 2015;87:882–884. doi:10.1038/ki.2015.60.

17. Moschcovitz: E. «An acute febrile pleiochromic anemia with hyaline thrombosis of terminal arteriols and capillaries: An undescribed disease». Arch Intern Med 36: 89. 1925

18. Gasser C, Gautier E, Tek A, Siebenmann E, Oechslin R. Haemolytisch urämische Syndrome: bilaterale Nierenrindennekrosen bei akuten erworbenen haemolytischen Anemien. Schweiz Med Wochenschr 1955; 85: 905-9.
No national or foreign publication may partially or totally reproduce or translate Revista Colombiana de Nefrología articles or abstracts without prior written permission from the journal’s Editorial Board.




Download data is not yet available.