medigraphic.com

2023, Number 2

<< Back Next >>

Rev Hematol Mex 2023; 24 (2)

Predictive value of diagnostic scores of thrombotic thrombocytopenic purpura and its clinical application

Ramírez AJM, Alonso GD, Martínez VMI, Anaya CJ, Borjas GC

Language: Spanish
References: 45
Page: 79-88
PDF size: 261.26 Kb.


ABSTRACT

Immune thrombotic thrombocytopenic purpura is included within the spectrum of thrombotic microangiopathies, it is recognized as a hematological emergency since it carries a potentially fatal risk due to secondary organ damage. Different clinical and laboratory variables have been described to increase the suspicion of this disease; however, the definitive diagnosis requires the study of ADAMTS13, as well as the measurement of its IgG inhibitory antibody. Due to its limited availability, different predictive scales of severe ADAMTS13 deficiency have been developed. This article presents a review of the development of different diagnostic scales, a comparison among them, their utility and application in daily practice, usefulness of scales as a guide in management decisions, as well as a brief review of current diagnostic tools.


REFERENCES

  1. Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B,Iorio A, Matsumoto M, Mustafa RA, Pai M, Rock G, RussellL, Tarawneh R, Valdes J, Peyvandi F. ISTH guidelines forthe diagnosis of thrombotic thrombocytopenic purpura. JThromb Haemost 2020; 18 (10): 2486-2495. doi: 10.1111/jth.15006.

  2. Moschcowitz E. Hyaline thrombosis of the terminal arteriolesand capillaries: a hitherto undescribed disease. ProcN Pathol Soc 1924; 24: 21-24.

  3. Sukumar S, Lämmle B, Cataland SR. Thrombotic thrombocytopenicpurpura: pathophysiology, diagnosis, andmanagement. J Clin Med 2021; 10 (3): 536. doi: 10.3390/jcm10030536.

  4. Moake JL, Rudy CK, Troll JH, Weinstein MJ, et al. Unusuallylarge plasma factor VIII:von Willebrand factor multimers inchronic relapsing thrombotic thrombocytopenic purpura.N Engl J Med 1982; 307 (23): 1432-1435. doi: 10.1056/NEJM198212023072306.

  5. Furlan M, Robles R, Lämmle B. Partial purification andcharacterization of a protease from human plasma cleavingvon Willebrand factor to fragments produced by in vivoproteolysis. Blood 1996; 87 (10): 4223-4234.

  6. Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenicpurpura. Blood 2017; 129 (21): 2836-2846. doi:10.1182/blood-2016-10-709857.

  7. Levy GG, Nichols WC, Lian EC, Foroud T, et al. Mutations ina member of the ADAMTS gene family cause thromboticthrombocytopenic purpura. Nature 2001; 413 (6855): 488-494. doi: 10.1038/35097008.

  8. Joly B, Stepanian A, Hajage D, Thouzeau S, et al. Evaluationof a chromogenic commercial assay using VWF-73 peptidefor ADAMTS13 activity measurement. Thromb Res 2014;134: 1074-1080. doi: 10.1016/j.thromres.2014.09.006.

  9. Alvarez-Larrán A, del Río-Garma J, Pujol M, de la RubiaJ, Hernández-Jodra M, Borrell M, et al. Newly diagnosedversus relapsed idiopathic thrombotic thrombocytopenicpurpura: A comparison of presenting clinical characteristicsand response to treatment. Ann Hematol 2009; 88: 973-8.doi: 10.1007/s00277-009-0707-9.

  10. Mariotte E, Azoulay E, Galicier L, Rondeau E, et al; FrenchReference Center for Thrombotic Microangiopathies.Epidemiology and pathophysiology of adulthood-onsetthrombotic microangiopathy with severe ADAMTS13deficiency (thrombotic thrombocytopenic purpura): across-sectional analysis of the French national registryfor thrombotic microangiopathy. Lancet Haematol 2016;3 (5): e237-e245. doi: 10.1016/S2352-3026(16)30018-7.

  11. Blombery P, Kivivali L, Pepperell D, McQuilten Z, et al; TTPregistry steering committee. Diagnosis and managementof thrombotic thrombocytopenic purpura (TTP) in Australia:findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry. Intern Med J 2016;46 (1): 71-79. doi: 10.1111/imj.12935.

  12. Hanna RM, Henriksen K, Kalantar-Zadeh K, Ferrey A,Burwick R, Jhaveri KD. Thrombotic microangiopathysyndromes-common ground and distinct frontiers. AdvChronic Kidney Dis 2022; 29 (2): 149-160. doi: 10.1053/j.ackd.2021.11.006.

  13. Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD,Kremer Hovinga J, Lammle B, Matsumoto M, Pavenski K,Sadler E, Sarode R, Wu H; International Working Groupfor Thrombotic Thrombocytopenic P. Consensus on thestandardization of terminology in thrombotic thrombocytopenicpurpura and related thrombotic microangiopathies.J Thromb Haemost 2016; 15: 312-22. doi: 10.1111/jth.13571.

  14. Sadler JE. What’s new in the diagnosis and pathophysiologyof thrombotic thrombocytopenic purpura. HematologyAm Soc Hematol Educ Program. 2015; 2015: 631-636. doi:10.1182/asheducation-2015.1.631.

  15. Saha M, McDaniel JK, Zheng XL. Thrombotic thrombocytopenicpurpura: pathogenesis, diagnosis and potential noveltherapeutics. J Thromb Haemost 2017; 15 (10): 1889-1900.doi: 10.1111/jth.13764.

  16. Kessler CS, Khan BA, Lai-Miller K. Thrombotic thrombocytopenicpurpura: A hematological emergency. J Emerg Med2012; 43: 538-44. doi: 10.1016/j.jemermed.2012.01.027.

  17. Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, PeyvandiF, et al. Guidelines on the diagnosis and management ofthrombotic thrombocytopenic purpura and other thromboticmicroangiopathies. Br J Haematol 2012; 158: 323-35.doi: 10.1111/j.1365-2141.2012.09167.x.

  18. Rogers HJ, Allen C, Lichtin AE. Thrombotic thrombocitopenicpurpura: The role of ADAMTS13.Cleve Clin J Med2016; 83 (8): 597-603.

  19. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect ofplasma exchange on plasma ADAMTS13 metalloproteaseactivity, inhibitor level, and clinical outcome in patientswith idiopathic and nonidiopathic thrombotic thrombocytopenicpurpura. Blood 2004; 103 (11): 4043-4049. doi:10.1182/blood-2003-11-4035.

  20. Barrows BD, Teruya J. Use of the ADAMTS13 activity assayimproved the accuracy and efficiency of the diagnosis andtreatment of suspected acquired thrombotic thrombocytopenicpurpura. Arch Pathol Lab Med 2014; 138: 546-9. doi:10.5858/arpa.2013-0170-OA.

  21. Bendapudi PK, Li A, Hamdan A, Uhl L, et al. Impact of severeADAMTS13 deficiency on clinical presentation and outcomesin patients with thrombotic microangiopathies: theexperience of the Harvard TAM Research Collaborative. BrJ Haematol 2015; 171 (5): 836-44. doi: 10.1111/bjh.13658.

  22. Shah N, Rutherford C, Matevosyan K, Shen YM, SarodeR. Role of ADAMTS13 in the management of thromboticmicroangiopathies including thrombotic thrombocytopenicpurpura (TTP). Br J Haematol 2013; 163 (4): 514-9. doi:10.1111/bjh.12569.

  23. Contreras E, de la Rubia J, Del Río-Garma J, Diaz-Ricart M,et al. Diagnostic and therapeutic guidelines of thromboticmicroangiopathies of the Spanish Apheresis Group. MedClin (Barc) 2015; 144 (7): 331.e1-331.e13. doi: 10.1016/j.medcli.2014.09.013.

  24. Zhou Z, Nguyen TC, Guchhait P, Dong JF. Von Willebrandfactor, ADAMTS-13, and thrombotic thrombocytopenicpurpura. Semin Thromb Hemost 2010; 36 (1): 71-81.https://doi.org/10.1182/blood-2008-02-078170.

  25. Kostousov V, Fehr J, Bombeli T. Novel semi-automated,60-min assay to determine von Willebrand factor cleavingactivity of ADAMTS13.Thromb Res 2006; 118 (6): 723-31.doi: 10.1016/j.thromres.2005.12.006.

  26. Jin M, Cataland S, Bissell M, Wu HM. A rapid test for dediagnosis of thrombotic thrombocytoprnic purpura usingsurface enhanced laser desorption/ionization time-of-flight(SELDI-TOF)-mass spectrometry. J Thromb Haemost 2006;4 (2): 333-8. doi: 10.1111/j.1538-7836.2006.01758.x.

  27. Groot E, Hulstein JJ, Rison CN, de Groot PG, Fijnheer R.FRETS- VWF73: a rapid and predictive tool for thromboticthrombocytopenic purpura. J Thromb Haemost 2006; 4:698-699. doi: 10.1111/j.1538-7836.2005.01767.x.

  28. Franchini M, Mannucci PM. Advantages and limits of ADAMTS13testing in thrombotic thrombocytopenic purpura.Blood Transfus 2008; 6: 127-135. doi: 10.2450/2008.0056-07.

  29. George JN, Nester CM. Syndromes of thrombotic microangiopathy.N Engl J Med 2014; 371: 654-66. DOI: 10.1056/NEJMra1312353.

  30. Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN.Survival and relapse in patients with thrombotic thrombocytopenicpurpura. Blood 2010; 115: 1500-1511. doi:10.1182/blood-2009-09-243790.

  31. Rose M, Eldor A. High incidence of relapses in thromboticthrombocytopenic purpura: clinical study of 38 patients.Am J Med 1987; 83 (3): 437-44. doi: 10.1016/0002-9343(87)90753-4.

  32. Wyllie BF, Garg AX, Macnab J, Rock GA, Clark WF, CanadianApheresis Group. Thrombotic thrombocytopenic purpura/haemolytic uraemic syndrome: a new index predictingresponse to plasma exchange. Br J Haematol 2006; 132 (2):204-9. doi: 10.1111/j.1365-2141.2005.05857.x.

  33. Benhamou Y, Assie C, Boelle PY, Buffet M, et al. Developmentand validation of a predictive model for death inacquired severe ADAMTS13 deficiency-associated idiopathicthrombotic thrombocytopenic purpura: the FrenchTMA Reference Center experience. Haematologica 2012;97: 1181-1186. doi: 10.3324/haematol.2011.049676.

  34. Coppo P, Schwarzinger M, Buffet M, Wynckel A, ClabaultK, et al. Predictive features of severe acquired ADAMTS13deficiency in idiopathic thrombotic microangiopathies:The French TMA Reference Center Experience. PLoS One2010; 5 (4): e10208. doi: 10.1371/journal.pone.0010208.

  35. Bendapudi PK, Makar RS. An algorithmic approach to thediagnosis and management of the thrombotic microangiopathies.Am J Clin Pathol 2016; 145 (2): 152-4. https://doi.org/10.1093/ajcp/aqw003.

  36. Bendapudi PK, Hurwitz S, Fry A, Marques MB, et al. Derivationand external validation of the PLASMIC score for rapidassessment of adults with thrombotic microangiopathies:a cohort study. Lancet Haematol 2017; 4: e157-e164. doi:10.1016/S2352-3026(17)30026-1.

  37. Bendapudi PK, Li A, Hamdan A, Fry A, et al. Derivationand prospective validation of a predictive score for therapid diagnosis of thrombotic thrombocytopenic purpura:the PLASMIC score. Blood 2014; 124: 231. https://doi.org/10.1182/blood.V124.21.231.231.

  38. Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnosticaccuracy of the PLASMIC score in patients with suspectedthrombotic thrombocytopenic purpura: A systematicreview and meta-analysis. Transfusion 2020; 60 (9): 2047-2057. https://doi.org/10.1111/trf.15954.

  39. Williams LA, Marques MB. Pathology consultation on thdiagnosis and of treatment of thrombotic microangiopaties(TMAs). Am J Clin Pathol 2016; 145 (2): 158-65. doi:10.1093/ajcp/aqv086.

  40. Li A, Khalighi PR, Wu Q, Garcia DA. External validation ofthe PLASMIC score: a clinical prediction tool for thromboticthrombocytopenic purpura diagnosis and treatment. JThromb Haemost 2018; 16: 164-9. doi: 10.1111/jth.13882.

  41. Liu A, Dhaliwal N, Upreti H, Kasmani J, Dane K, Moliterno A,Braunstein E, Brodsky R, Chaturvedi S. Reduced sensitivityof PLASMIC and French scores for the diagnosis of thromboticthrombocytopenic purpura in older individuals. Transfusion2021; 61 (1). https://doi.org/10.1111/trf.16188.

  42. Tiscia GL, Ostuni A, Cascavilla N, Cappucci F, et al. Validationof PLASMIC score and follow-up data in a cohort of patientswith suspected microangiopathies from Southern Italy. JThromb Thrombolysis 2018; 46: 174-179. doi: 10.1007/s11239-018-1674-6.

  43. Gavriilaki E, Koravou E, Chatziconstantinou T, Kalpadakis C,et al. Plasmic and plasic αre εxcellent predictors of severeADAMTS13 deficiency in thrombotic microangiopathypatients without secondary causes. Blood 2019; 134 (1):4913. https://doi.org/10.1182/blood-2019-125343.

  44. Upadhyay VA, Geisler BP, Sun L, Uhl L, et al. Utilizing aPLASMIC score- based approach in the management ofsuspected immune thrombotic thrombocytopenic purpura:a cost minimization analysis within the Harvard TMAResearch Collaborative. Br J Haematol 2019; 186: 490-498.doi: 10.1111/bjh.15932.

  45. Coelho DP, Marques BA, Roque A, Carda JP, Martinho P,Fidalgo T, Ribeiro ML. Comparative analysis of clinicalprediction scores in acquired thrombotic thrombocytopenicpurpura: The superiority of plasmic score. Blood2020; 136 (Supplement 1). https://doi.org/10.1182/blood-2020-143159.




2020     |     www.medigraphic.com