Cómo citar
1.
Ramírez JI, Gómez ED, Aldana Campos MC, Puerto R, Córdoba JP. Síndrome hemolítico urémico atípico posterior a trasplante renal: presentación de un caso y revisión de la literatura. Rev. Colomb. Nefrol. [Internet]. 1 de enero de 2017 [citado 3 de julio de 2024];4(1):74-8. Disponible en: https://revistanefrologia.org/index.php/rcn/article/view/267
Resumen
El síndrome hemolítico urémico (SHU) es una entidad clínica caracterizada por la aparición de anemia hemolítica no inmune, trombocitopenia e insuficiencia renal aguda. Se trata de una enfermedad perteneciente al grupo de las microangiopatías trombóticas (MAT) de la que hacen parte también la purpura trombocitopénica trombótica (PTT) y algunas otras MAT asociadas a otras condiciones médicas antes conocidas como MAT secundarias.
Por otra parte, la variedad conocida como SHU atípico (SHUa) es una patología ultra-huérfana que frecuentemente evoluciona a insuficiencia renal crónica (IRC) y se asocia con elevada morbimortalidad si no recibe el tratamiento adecuado.
Se examina el caso de un paciente que presenta su primera manifestación clínica de síndrome hemolítico urémico atípico después de trasplante renal cadavérico lo cual no solo lo hace un caso aún más exótico, sino que implica mayor complejidad en su manejo.
Citas
1. George JN, Charania RS. Evaluation of patients with microangiopathic hemolytic anemia and thrombocytopenia. Semin Thromb Hemost [Internet]. 2013 [accedido 01 Jan 2000];39(2):153–60. Available from: http://dx.doi.org/10.1055/s-0032-1333538. Mayo 2016.
2. Polito MG, Kirsztajn GM. Thrombotic microangiopathies: thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. J Bras Nefrol [Internet]. 2010 [accedido 01 Jan 2000];32(3):303–15. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21103695\nhttp://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=21103695. Mayo 2016.
3. Campistol Plana JM, Arias M, Ariceta Iraola G, Blasco M, Espinosa M, Grinyo JM, et al. Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Nefrología (Madr.) [Internet]. 2013 [accedido 01 Jan 2000];33:27–45.
Available from: http://dx.doi.org/10.3265Nefrologia.pre2012.Nov.11781
4. Cordoba JP, Mariel K, Carolina C, Espitaleta Z, Gonzalez E, Ibarra M, et al. Síndrome hemolítico urémico atípico, revisión de la literatura y documento de consenso: enfoque diagnóstico y tratamiento.Rev Colomb Nefrol. 2015;2(1):19–40.
5. Kavanagh D, Goodship THJ. Atypical hemolytic uremic syndrome. Curr Opin Hematol. 2010;17(5):432–8.
6. Zipfel PF, Wolf G, Jhon U, Kentouche K, and Skerka C. Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura? Pediatr Nephrol. 2011;26: 1947-56.
7. Ali M, Syed A, Bhandari S. Case series: hemolytic uremic syndrome another cause of transplant dysfunction. Transplant Proc [Internet]. 2013 [accedido 01 Jan 2000];45(9):3284–8.
Available from:http://dx.doi.org/10.1016/j.transproceed.2013.07.060
8. Hirt-Minkowski P, Dickenmann M, Schifferli JA. Atypical Hemolytic Uremic Syndrome: Update on the Complement System and What Is New. Nephron Clin Pract [Internet]. 2010 [accedido 01 Jan2000];114(4):c219–35.
Available from: http://dx.doi.org/10.1159/000276545
9. Laurence J. Atypical Hemolytic Uremic Syndrome ( aHUS ): making the diagnosis. Clin Adv Hematol Oncol [Internet]. 2012 Oct [accedido 01 Jan 2000]; 10 (10 Suppl 17):1-12.
Available from: https://www.ncbi.nlm.nih.gov/pubmed/23187605
10. Kreuter J, Winters JL. Drug-associated thrombotic microangiopathies. Semin Thromb Hemost [Internet]. 2012 [accedido 01 Jan 2000];38(8):839–44.
Available from: http://dx.doi.org/10.1055/s-0032-1328886
11. Vega J, Parodi C, Mendez GP, Goecke H. Síndrome hemolítico-urémico asociado al uso de gemcitabina. Rev Med Chil. 2013;141(6):797–802.
12. Fakhouri F, Fremeaux-Bacchi V, Loirat C. Atypical hemolytic uremic syndrome: from the rediscovery of complement to targeted therapy. Eur J Intern Med [Internet]. 2013 [accedido 01 Jan 2000];24(6):492–5. Available from: http://dx.doi.org/10.1016/j.ejim.2013.05.008
13. de Cordoba SR, Montes T. Síndrome hemolítico urémico atípico. Nefrol Supl Extraordin. 2011;2(1):58–65.
14. Joseph C, Gattineni J. Complement disorders and hemolytic uremic syndrome. Curr Opin Pediatr [Internet]. 2013 Apr [accedido 01 Jan 2000];25(2):209–15.
Available from: http://dx.doi.org/10.1097/MOP.0b013e32835df48a
15. Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic uremic syndrome. N Engl J Med [Internet]. 2013 [accedido 01 Jan 2000];368(23):2169–81.
Available from: http://dx.doi.org/10.1056/NEJMoa1208981
16. Rathbone J, Kaltenthaler E, Richards A, Tappenden P, Bessey A, Cantrell A. A systematic review of eculizumab for atypical haemolytic uraemic syndrome (aHUS). BMJ Open [Internet]. 2013 [accedido 01 Jan 2000];3(11): e003573.
Available from: http://dx.doi.org/10.1136/bmjopen-2013-003573
17. Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis [Internet]. 2011 [accedido 01 Jan 2000];6(1):60. Available from: http://dx.doi.org/10.1186/1750-1172-6-60
18. Kavanagh D, Goodship T. Genetics and complement in atypical HUS. Pediatr Nephrol [Internet]. 2010 [accedido 01 Jan 2000];25(12):2431–42. Available from: http://dx.doi.org/10.1007s00467-010-1555-5
19. Kremer Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood [Internet]. 2010 [accedido 01 Jan 2000];115(8):1500-11.
Available from http://dx.doi.org/10.1182/blood-2009-09-243790
20. Keating GM. Eculizumab: A Review of Its Use in Atypical Haemolytic Uraemic Syndrome. Drugs [Internet]. 2013 [accedido en 01 Jan 2000];73(18):2053–66.
Available from: http://dx.doi.org/10.1007/s40265-013-0147-7
21. Licht C, Greenbaum LA, Muus P, Babu S, Bedrosian CL, Cohen DJ, Delmas Y, et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int [Internet]. 2015 [accedido 01 Jan 2000]; 87(5):1061-1073.
Available from: http://dx.doi.org/10.1038/ki.2014.423
22. Hillmen P, Muus P, Roth A, Elebute MO, Risitano AM, Schrezenmeier H, et al. Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol [Internet]. 2013 [accedido 01 Jan 2000];162(1):62–73.
Available from: http://dx.doi.org/10.1111/bjh.12347
23. Rother RP, Rollins SA, Mojcik CF, Brodsky RA, Bell L. Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria. Nat Biotechnol [Internet]. 2007 [accedido 01 Jan 2000];25(11):1256–64.
Available from: http://dx.doi.org/10.1038/nbt1344
24. Hillmen P. The role of complement inhibition in PNH. Hematology [Internet]. 2008 [accedido 01 Jan 2000];2008(1):116–23.
Available from: https://doi.org/10.1182/asheducation-2008.1.116
25. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher M A, Niaudet P, Guest G, et al. Differential Impact of Complement Mutations on Clinical Characteristics in Atypical Hemolytic Uremic Syndrome. J Am Soc Nephrol [Internet]. 2007 [accedido 01 Jan 2000];18(8):2392–400. Available from: https://doi.org/10.1681/ASN.2006080811
26. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, et al. Genetics of HUS : the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood [Internet]. 2006 [accedido 01 Jan 2000];108(4):1267–79.
Available from: https://doi.org/10.1182/blood-2005-10-007252.
2. Polito MG, Kirsztajn GM. Thrombotic microangiopathies: thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. J Bras Nefrol [Internet]. 2010 [accedido 01 Jan 2000];32(3):303–15. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21103695\nhttp://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=21103695. Mayo 2016.
3. Campistol Plana JM, Arias M, Ariceta Iraola G, Blasco M, Espinosa M, Grinyo JM, et al. Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Nefrología (Madr.) [Internet]. 2013 [accedido 01 Jan 2000];33:27–45.
Available from: http://dx.doi.org/10.3265Nefrologia.pre2012.Nov.11781
4. Cordoba JP, Mariel K, Carolina C, Espitaleta Z, Gonzalez E, Ibarra M, et al. Síndrome hemolítico urémico atípico, revisión de la literatura y documento de consenso: enfoque diagnóstico y tratamiento.Rev Colomb Nefrol. 2015;2(1):19–40.
5. Kavanagh D, Goodship THJ. Atypical hemolytic uremic syndrome. Curr Opin Hematol. 2010;17(5):432–8.
6. Zipfel PF, Wolf G, Jhon U, Kentouche K, and Skerka C. Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura? Pediatr Nephrol. 2011;26: 1947-56.
7. Ali M, Syed A, Bhandari S. Case series: hemolytic uremic syndrome another cause of transplant dysfunction. Transplant Proc [Internet]. 2013 [accedido 01 Jan 2000];45(9):3284–8.
Available from:http://dx.doi.org/10.1016/j.transproceed.2013.07.060
8. Hirt-Minkowski P, Dickenmann M, Schifferli JA. Atypical Hemolytic Uremic Syndrome: Update on the Complement System and What Is New. Nephron Clin Pract [Internet]. 2010 [accedido 01 Jan2000];114(4):c219–35.
Available from: http://dx.doi.org/10.1159/000276545
9. Laurence J. Atypical Hemolytic Uremic Syndrome ( aHUS ): making the diagnosis. Clin Adv Hematol Oncol [Internet]. 2012 Oct [accedido 01 Jan 2000]; 10 (10 Suppl 17):1-12.
Available from: https://www.ncbi.nlm.nih.gov/pubmed/23187605
10. Kreuter J, Winters JL. Drug-associated thrombotic microangiopathies. Semin Thromb Hemost [Internet]. 2012 [accedido 01 Jan 2000];38(8):839–44.
Available from: http://dx.doi.org/10.1055/s-0032-1328886
11. Vega J, Parodi C, Mendez GP, Goecke H. Síndrome hemolítico-urémico asociado al uso de gemcitabina. Rev Med Chil. 2013;141(6):797–802.
12. Fakhouri F, Fremeaux-Bacchi V, Loirat C. Atypical hemolytic uremic syndrome: from the rediscovery of complement to targeted therapy. Eur J Intern Med [Internet]. 2013 [accedido 01 Jan 2000];24(6):492–5. Available from: http://dx.doi.org/10.1016/j.ejim.2013.05.008
13. de Cordoba SR, Montes T. Síndrome hemolítico urémico atípico. Nefrol Supl Extraordin. 2011;2(1):58–65.
14. Joseph C, Gattineni J. Complement disorders and hemolytic uremic syndrome. Curr Opin Pediatr [Internet]. 2013 Apr [accedido 01 Jan 2000];25(2):209–15.
Available from: http://dx.doi.org/10.1097/MOP.0b013e32835df48a
15. Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic uremic syndrome. N Engl J Med [Internet]. 2013 [accedido 01 Jan 2000];368(23):2169–81.
Available from: http://dx.doi.org/10.1056/NEJMoa1208981
16. Rathbone J, Kaltenthaler E, Richards A, Tappenden P, Bessey A, Cantrell A. A systematic review of eculizumab for atypical haemolytic uraemic syndrome (aHUS). BMJ Open [Internet]. 2013 [accedido 01 Jan 2000];3(11): e003573.
Available from: http://dx.doi.org/10.1136/bmjopen-2013-003573
17. Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis [Internet]. 2011 [accedido 01 Jan 2000];6(1):60. Available from: http://dx.doi.org/10.1186/1750-1172-6-60
18. Kavanagh D, Goodship T. Genetics and complement in atypical HUS. Pediatr Nephrol [Internet]. 2010 [accedido 01 Jan 2000];25(12):2431–42. Available from: http://dx.doi.org/10.1007s00467-010-1555-5
19. Kremer Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood [Internet]. 2010 [accedido 01 Jan 2000];115(8):1500-11.
Available from http://dx.doi.org/10.1182/blood-2009-09-243790
20. Keating GM. Eculizumab: A Review of Its Use in Atypical Haemolytic Uraemic Syndrome. Drugs [Internet]. 2013 [accedido en 01 Jan 2000];73(18):2053–66.
Available from: http://dx.doi.org/10.1007/s40265-013-0147-7
21. Licht C, Greenbaum LA, Muus P, Babu S, Bedrosian CL, Cohen DJ, Delmas Y, et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int [Internet]. 2015 [accedido 01 Jan 2000]; 87(5):1061-1073.
Available from: http://dx.doi.org/10.1038/ki.2014.423
22. Hillmen P, Muus P, Roth A, Elebute MO, Risitano AM, Schrezenmeier H, et al. Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol [Internet]. 2013 [accedido 01 Jan 2000];162(1):62–73.
Available from: http://dx.doi.org/10.1111/bjh.12347
23. Rother RP, Rollins SA, Mojcik CF, Brodsky RA, Bell L. Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria. Nat Biotechnol [Internet]. 2007 [accedido 01 Jan 2000];25(11):1256–64.
Available from: http://dx.doi.org/10.1038/nbt1344
24. Hillmen P. The role of complement inhibition in PNH. Hematology [Internet]. 2008 [accedido 01 Jan 2000];2008(1):116–23.
Available from: https://doi.org/10.1182/asheducation-2008.1.116
25. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher M A, Niaudet P, Guest G, et al. Differential Impact of Complement Mutations on Clinical Characteristics in Atypical Hemolytic Uremic Syndrome. J Am Soc Nephrol [Internet]. 2007 [accedido 01 Jan 2000];18(8):2392–400. Available from: https://doi.org/10.1681/ASN.2006080811
26. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, et al. Genetics of HUS : the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood [Internet]. 2006 [accedido 01 Jan 2000];108(4):1267–79.
Available from: https://doi.org/10.1182/blood-2005-10-007252.
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