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2021, Número 2

Medicina & Laboratorio 2021; 25 (2)


Microangiopatías trombóticas primarias: una revisión narrativa

Henao-Piedrahita N
Texto completo Cómo citar este artículo

Idioma: Español
Referencias bibliográficas: 63
Paginas: 485-499
Archivo PDF: 117.01 Kb.


PALABRAS CLAVE

microangiopatías trombóticas, púrpura trombocitopénica trombótica, síndrome hemolítico urémico, síndrome hemolítico urémico atípico, anemia hemolítica, trombocitopenia.

RESUMEN

La microangiopatía trombótica (MAT) es un síndrome donde hay formación de microtrombos en la circulación que llevan a anemia hemolítica microangiopática (AHMA) y trombocitopenia con falla multiorgánica, debido a la isquemia de los tejidos. Las MAT pueden ser primarias sin causa subyacente asociada, como la púrpura trombocitopénica trombótica debida a deficiencia de la enzima ADAMTS13, el síndrome hemolítico urémico debido a la toxina Shiga de Escherichia coli enterohemorrágica, y la MAT producida por alteraciones en la regulación del complemento. Adicionalmente, pueden ser secundarias a enfermedades malignas, infecciosas, metabólicas, autoinmunes o inducidas por el embarazo. Estas patologías requieren diagnóstico y tratamiento oportunos debido a que tienen alta morbimortalidad y se asocian a complicaciones que incluyen enfermedad renal, alteraciones neurológicas como convulsiones, accidente cerebrovascular, coma y muerte. El tratamiento es multidisciplinario y se enfoca en el soporte hemodinámico, transfusional y en el manejo de la etiología cuando esta es identificada. La siguiente revisión pretende explicar de forma clara y precisa los aspectos generales de las MAT primarias.


REFERENCIAS (EN ESTE ARTÍCULO)

  1. Moschcowitz E. An acute febrile pleiochromicanemia with hyaline thrombosis of the terminalarterioles and capillaries; an undescribed disease.Am J Med 1952;13:567-569. https://doi.org/10.1016/0002-9343(52)90022-3.

  2. Contreras E, De la Rubia J, del Río-GarmaJ, Díaz-Ricart M, García-Gala JM, Lozano M.Guía diagnóstica y terapéutica de las microangiopatíastrombóticas del Grupo Español deAféresis. Med Clin 2015;144:e1-e13. https://doi.org/10.1016/j.medcli.2014.09.013.

  3. Arnold DM, Patriquin CJ, Nazy I. Thromboticmicroangiopathies: a general approach to diagnosisand management. CMAJ 2017;189:E153-E159. https://doi.org/10.1503/cmaj.160142.

  4. Radhakrishnan J. Anticomplement therapies in“secondary thrombotic microangiopathies”: readyfor prime time? Kidney Int 2019;96:833-835.https://doi.org/10.1016/j.kint.2019.08.005.

  5. George JN, Nester CM. Syndromes ofthrombotic microangiopathy. N Engl J Med2014;371:654-666. https://doi.org/10.1056/NEJMra1312353.

  6. Moake JL. Thrombotic microangiopathies.N Engl J Med 2002;347:589-600. https://doi.org/10.1056/NEJMra020528.

  7. Romero S, Sempere A, Gómez-Seguí I, RománE, Moret A, Jannone R, et al. Guía prácticade tratamiento urgente de la microangiopatíatrombótica. Med Clin 2018;151:123.e121-123.e129. https://doi.org/https://doi.org/10.1016/j.medcli.2018.01.013.

  8. George JN, Nester CM. Approach to the patientwith suspected TTP, HUS, or other thromboticmicroangiopathy (TMA). Massachusetts,Estados Unidos: Wolters Kluwer Health; 2019.Acceso 20 de agosto de 2020. Disponible enhttps://www.uptodate.com/contents/approachto-the-patient-with-suspected-ttp-hus-or-otherthrombotic-microangiopathy-tma#H16673902.

  9. Nokes T, George JN, Vesely SK, AwabA. Pulmonary involvement in patients withthrombotic thrombocytopenic purpura. EurJ Haematol 2014;92:156-163. https://doi.org/10.1111/ejh.12222.

  10. Blasco-Pelicano M, Guillé E, Quintana L, Garcia-Herrera A, Piñeiro G, Poch E, et al. Thromboticmicroangiopathies assessment: mind thecomplement. Clin Kidney J 2020;2020:sfaa195.https://doi.org/10.1093/ckj/sfaa195.

  11. Eskazan AE, Salihoglu A. Treatment and outcomeof primary and secondary thrombotic microangiopathies.Am J Nephrol 2015;41:427-428. https://doi.org/10.1159/000437002.

  12. Kremer Hovinga JA, Coppo P, LämmleB, Moake JL, Miyata T, Vanhoorelbeke K.Thrombotic thrombocytopenic purpura. NatRev Dis Primers 2017;3:17020. https://doi.org/10.1038/nrdp.2017.20.

  13. Joly BS, Coppo P, Veyradier A. An updateon pathogenesis and diagnosis of thromboticthrombocytopenic purpura. Expert Rev Hematol2019;12:383-395. https://doi.org/10.1080/17474086.2019.1611423.

  14. Shatzel JJ, Taylor JA. Syndromes of thromboticmicroangiopathy. Med Clin North Am2017;101:395-415. https://doi.org/10.1016/j.mcna.2016.09.010.

  15. George JN. Thrombotic microangiopathy(TMA). Platelets on the Web. Oklahoma, USA:University of Oklahoma Health Sciences Center;2007. Acceso 5 de agosto de 2020. Disponibleen https://www.ouhsc.edu/platelets/TMA.htm.

  16. Reese JA, Muthurajah DS, Kremer HovingaJA, Vesely SK, Terrell DR, George JN. Children and adults with thrombotic thrombocytopenicpurpura associated with severe, acquiredAdamts13 deficiency: comparison of incidence,demographic and clinical features. PediatrBlood Cancer 2013;60:1676-1682. https://doi.org/10.1002/pbc.24612.

  17. Terrell DR, Williams LA, Vesely SK, Lämmle B,Hovinga JA, George JN. The incidence of thromboticthrombocytopenic purpura-hemolytic uremicsyndrome: all patients, idiopathic patients,and patients with severe ADAMTS-13 deficiency.J Thromb Haemost 2005;3:1432-1436. https://doi.org/10.1111/j.1538-7836.2005.01436.x.

  18. Terrell DR, Vesely SK, Kremer Hovinga JA,Lämmle B, George JN. Different disparities ofgender and race among the thrombotic thrombocytopenicpurpura and hemolytic-uremicsyndromes. Am J Hematol 2010;85:844-847.https://doi.org/10.1002/ajh.21833.

  19. Moake JL, Rudy CK, Troll JH, Weinstein MJ,Colannino NM, Azocar J, et al. Unusuallylarge plasma factor VIII:von Willebrand factormultimers in chronic relapsing thromboticthrombocytopenic purpura. N Engl J Med1982;307:1432-1435. https://doi.org/10.1056/nejm198212023072306.

  20. Asada Y, Sumiyoshi A, Hayashi T, Suzumiya J,Kaketani K. Immunohistochemistry of vascularlesion in thrombotic thrombocytopenic purpura,with special reference to factor VIII relatedantigen. Thromb Res 1985;38:469-479. https://doi.org/10.1016/0049-3848(85)90180-x.

  21. Vacca VM, Jr. Acquired autoimmune thromboticthrombocytopenic purpura. Nursing2019;49:22-29. https://doi.org/10.1097/01.NURSE.0000549721.69197.4d.

  22. Moake JL, Turner NA, Stathopoulos NA, NolascoLH, Hellums JD. Involvement of largeplasma von Willebrand factor (vWF) multimersand unusually large vWF forms derived fromendothelial cells in shear stress-induced plateletaggregation. J Clin Invest 1986;78:1456-1461. https://doi.org/10.1172/jci112736.

  23. Zander CB, Cao W, Zheng XL. ADAMTS13and von Willebrand factor interactions. CurrOpin Hematol 2015;22:452-459. https://doi.org/10.1097/moh.0000000000000169.

  24. Furlan M, Robles R, Galbusera M, Remuzzi G,Kyrle PA, Brenner B, et al. von Willebrand factorcleavingprotease in thrombotic thrombocytopenicpurpura and the hemolytic-uremic syndrome.N Engl J Med 1998;339:1578-1584. https://doi.org/10.1056/nejm199811263392202.

  25. Blombery P, Scully M. Management of thromboticthrombocytopenic purpura: current perspectives.J Blood Med 2014;5:15-23. https://doi.org/10.2147/jbm.S46458.

  26. Kappler S, Ronan-Bentle S, Graham A.Thrombotic microangiopathies (TTP, HUS,HELLP). Hematol Oncol Clin North Am2017;31:1081-1103. https://doi.org/10.1016/j.hoc.2017.08.010.

  27. Griffin D, Al-Nouri ZL, Muthurajah D, Ross JR,Ballard RB, Terrell DR, et al. First symptomsin patients with thrombotic thrombocytopenicpurpura: what are they and when do they occur?Transfusion 2013;53:235-237. https://doi.org/10.1111/j.1537-2995.2012.03934.x.

  28. George JN. How I treat patients with thromboticthrombocytopenic purpura: 2010. Blood2010;116:4060-4069. https://doi.org/10.1182/blood-2010-07-271445.

  29. Vesely SK, George JN, Lämmle B, StudtJD, Alberio L, El-Harake MA, et al. ADAMTS13activity in thrombotic thrombocytopenicpurpura-hemolytic uremic syndrome:relation to presenting features and clinicaloutcomes in a prospective cohort of 142patients. Blood 2003;102:60-68. https://doi.org/10.1182/blood-2003-01-0193.

  30. Brocklebank V, Wood KM, Kavanagh D.Thrombotic microangiopathy and the kidney.Clin J Am Soc Nephrol 2018;13:300-317.https://doi.org/10.2215/cjn.00620117.

  31. Bendapudi PK, Hurwitz S, Fry A, MarquesMB, Waldo SW, Li A, et al. Derivation and externalvalidation of the PLASMIC score for rapidassessment of adults with thrombotic microangiopathies:a cohort study. Lancet Haematol2017;4:e157-e164. https://doi.org/10.1016/s2352-3026(17)30026-1.

  32. George JN. Systemic malignancies as a causeof unexpected microangiopathic hemolyticanemia and thrombocytopenia. Oncology2011;25:908-914.

  33. Furlan M, Robles R, Morselli B, Sandoz P,Lämmle B. Recovery and half-life of von Willebrandfactor-cleaving protease after plasma therapy in patients with thrombotic thrombocytopenicpurpura. Thromb Haemost 1999;81:8-13.

  34. Zheng XL, Vesely SK, Cataland SR, CoppoP, Geldziler B, Iorio A, et al. ISTH guidelinesfor treatment of thrombotic thrombocytopenicpurpura. J Thromb Haemost 2020;18:2496-2502. https://doi.org/10.1111/jth.15010.

  35. Scully M, Hunt BJ, Benjamin S, Liesner R,Rose P, Peyvandi F, et al. Guidelines on thediagnosis and management of thromboticthrombocytopenic purpura and other thromboticmicroangiopathies. Br J Haematol2012;158:323-335. https://doi.org/10.1111/j.1365-2141.2012.09167.x.

  36. Schwartz J, Padmanabhan A, Aqui N, BalogunRA, Connelly-Smith L, Delaney M, et al.Guidelines on the use of therapeutic apheresisin clinical practice-evidence-based approachfrom the Writing Committee of the AmericanSociety for Apheresis: The seventh special issue.J Clin Apher 2016;31:149-162. https://doi.org/10.1002/jca.21470.

  37. Scully M, Cataland S, Coppo P, de la RubiaJ, Friedman KD, Kremer Hovinga J, et al.Consensus on the standardization of terminologyin thrombotic thrombocytopenic purpuraand related thrombotic microangiopathies. JThromb Haemost 2017;15:312-322. https://doi.org/10.1111/jth.13571.

  38. Wang HX, Han B, Zhao YY, Kou L, Guo LL,Sun TW, et al. Serum D-dimer as a potentialnew biomarker for prognosis in patients withthrombotic thrombocytopenic purpura. Medicine(Baltimore) 2020;99:e19563. https://doi.org/10.1097/md.0000000000019563.

  39. Gasser C, Gautier E, Steck A, SiebenmannRE, Oechslin R. [Hemolytic-uremic syndrome:bilateral necrosis of the renal cortex in acuteacquired hemolytic anemia]. Schweiz Med Wochenschr1955;85:905-909.

  40. Fakhouri F, Zuber J, Frémeaux-Bacchi V, LoiratC. Haemolytic uraemic syndrome. Lancet2017;390:681-696. https://doi.org/10.1016/s0140-6736(17)30062-4.

  41. Tarr PI, Gordon CA, Chandler WL. Shiga-toxinproducingEscherichia coli and haemolytic uraemicsyndrome. Lancet 2005;365:1073-1086.https://doi.org/10.1016/s0140-6736(05)71144-2.

  42. Witham PK, Yamashiro CT, Livak KJ, BattCA. A PCR-based assay for the detection of Escherichiacoli Shiga-like toxin genes in groundbeef. Appl Environ Microbiol 1996;62:1347-1353. https://doi.org/10.1128/aem.62.4.1347-1353.1996.

  43. Bell BP, Goldoft M, Griffin PM, Davis MA,Gordon DC, Tarr PI, et al. A multistate outbreakof Escherichia coli O157:H7-associatedbloody diarrhea and hemolytic uremic syndromefrom hamburgers. The Washington experience.Jama 1994;272:1349-1353.

  44. Buchholz U, Bernard H, Werber D, BöhmerMM, Remschmidt C, Wilking H, et al. Germanoutbreak of Escherichia coli O104:H4associated with sprouts. N Engl J Med2011;365:1763-1770. https://doi.org/10.1056/NEJMoa1106482.

  45. Noris M, Remuzzi G. Hemolytic uremic syndrome.J Am Soc Nephrol 2005;16:1035-1050.https://doi.org/10.1681/asn.2004100861.

  46. Cody EM, Dixon BP. Hemolytic uremic syndrome.Pediatr Clin North Am 2019;66:235-246.https://doi.org/10.1016/j.pcl.2018.09.011.

  47. Karpac CA, Li X, Terrell DR, Kremer HovingaJA, Lämmle B, Vesely SK, et al. Sporadic bloodydiarrhoea-associated thrombotic thrombocytopenicpurpura-haemolytic uraemic syndrome:an adult and paediatric comparison.Br J Haematol 2008;141:696-707. https://doi.org/10.1111/j.1365-2141.2008.07116.x.

  48. Manrique-Caballero CL, PeerapornratanaS, Formeck C, Del Rio-Pertuz G, Gomez DaniesH, Kellum JA. Typical and atypical hemolyticuremic syndrome in the critically ill.Crit Care Clin 2020;36:333-356. https://doi.org/10.1016/j.ccc.2019.11.004.

  49. Spinale JM, Ruebner RL, Copelovitch L, KaplanBS. Long-term outcomes of Shiga toxinhemolytic uremic syndrome. Pediatr Nephrol2013;28:2097-2105. https://doi.org/10.1007/s00467-012-2383-6.

  50. Garg AX, Suri RS, Barrowman N, Rehman F,Matsell D, Rosas-Arellano MP, et al. Longtermrenal prognosis of diarrhea-associatedhemolytic uremic syndrome: a systematic review,meta-analysis, and meta-regression. Jama2003;290:1360-1370. https://doi.org/10.1001/jama.290.10.1360.

  51. Taylor CM, Machin S, Wigmore SJ, GoodshipTH. Clinical practice guidelines for themanagement of atypical haemolytic uraemicsyndrome in the United Kingdom. Br J Haematol2010;148:37-47. https://doi.org/10.1111/j.1365-2141.2009.07916.x.

  52. Geerdink LM, Westra D, van Wijk JA, DorresteijnEM, Lilien MR, Davin JC, et al. Atypicalhemolytic uremic syndrome in children: complementmutations and clinical characteristics.Pediatr Nephrol 2012;27:1283-1291. https://doi.org/10.1007/s00467-012-2131-y.

  53. Hofer J, Janecke AR, Zimmerhackl LB, RiedlM, Rosales A, Giner T, et al. Complementfactor H-related protein 1 deficiency and factorH antibodies in pediatric patients withatypical hemolytic uremic syndrome. Clin JAm Soc Nephrol 2013;8:407-415. https://doi.org/10.2215/cjn.01260212.

  54. Licht C, Ardissino G, Ariceta G, Cohen D, ColeJA, Gasteyger C, et al. The global aHUS registry:methodology and initial patient characteristics.BMC Nephrol 2015;16:207. https://doi.org/10.1186/s12882-015-0195-1.

  55. Makou E, Herbert AP, Barlow PN. Functionalanatomy of complement factor H. Biochemistry2013;52:3949-3962. https://doi.org/10.1021/bi4003452.

  56. Noris M, Caprioli J, Bresin E, Mossali C, PianettiG, Gamba S, et al. Relative role of geneticcomplement abnormalities in sporadic andfamilial aHUS and their impact on clinical phenotype.Clin J Am Soc Nephrol 2010;5:1844-1859. https://doi.org/10.2215/cjn.02210310.

  57. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher MA, Niaudet P, GuestG, et al. Differential impact of complementmutations on clinical characteristics in atypicalhemolytic uremic syndrome. J Am Soc Nephrol2007;18:2392-2400. https://doi.org/10.1681/asn.2006080811.

  58. Goodship TH, Cook HT, Fakhouri F, FervenzaFC, Frémeaux-Bacchi V, Kavanagh D, et al. Atypicalhemolytic uremic syndrome and C3 glomerulopathy:conclusions from a "Kidney disease:Improving global outcomes" (KDIGO) ControversiesConference. Kidney Int 2017;91:539-551.https://doi.org/10.1016/j.kint.2016.10.005.

  59. Cataland SR, Holers VM, Geyer S, YangS, Wu HM. Biomarkers of terminal complementactivation confirm the diagnosis ofaHUS and differentiate aHUS from TTP. Blood2014;123:3733-3738. https://doi.org/10.1182/blood-2013-12-547067.

  60. Noris M, Remuzzi G. Atypical hemolytic-uremicsyndrome. N Engl J Med 2009;361:1676-1687.https://doi.org/10.1056/NEJMra0902814.

  61. Masias C, Vasu S, Cataland SR. None of the above:thrombotic microangiopathy beyond TTPand HUS. Blood 2017;129:2857-2863. https://doi.org/10.1182/blood-2016-11-743104.

  62. Gordon CE, Chitalia VC, Sloan JM, SalantDJ, Coleman DL, Quillen K, et al. Thromboticmicroangiopathy: A multidisciplinary teamapproach. Am J Kidney Dis 2017;70:715-721.https://doi.org/10.1053/j.ajkd.2017.05.017.

  63. Bittencourt CE, Ha JP, Maitta RW. Re-examinationof 30-day survival and relapse rates inpatients with thrombotic thrombocytopenicpurpura-hemolytic uremic syndrome. PLoS One2015;10:e0127744. https://doi.org/10.1371/journal.pone.0127744.




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Medicina & Laboratorio. 2021;25