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Semin Thromb Hemost 2017; 43(05): 540-548
DOI: 10.1055/s-0037-1603100
DOI: 10.1055/s-0037-1603100
Review Article
Clinical Scoring Systems in Thrombotic Microangiopathies
Authors
Weitere Informationen
Publikationsverlauf
Publikationsdatum:
08. Juni 2017 (online)
Abstract
Thrombotic microangiopathies (TMAs) are a group of rare but potentially lethal hematologic disorders characterized by platelet-rich thrombi in the microvasculature. In evaluating patients with TMA, it is crucial to identify those who have thrombotic thrombocytopenic purpura (TTP), a subtype of TMA that occurs due to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13). Individuals with TTP require urgent therapeutic plasma exchange, which is associated with a significant reduction in mortality; however, at most centers, the results of ADAMTS13 activity testing are usually not available for 3 to 5 days. Given the possible need for urgent intervention and the lack of timely test results to guide therapy, the assessment of patients with TMA represents an ideal situation for the deployment of diagnostic scoring systems to predict the presence of severe ADAMTS13 deficiency. Here, we review the literature surrounding clinical prediction tools in the diagnosis and prognostication of patients with TMA, describe the experience at our center with evaluating TMA patients, and discuss the utility of clinical scoring systems for TMA in the context of patient care.
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References
- 1 George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014; 371 (07) 654-666
- 2 Bendapudi PK, Li A, Hamdan A. , et al. Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative. Br J Haematol 2015; 171 (05) 836-844
- 3 Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87 (10) 4223-4234
- 4 Furlan M, Robles R, Galbusera M. , et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998; 339 (22) 1578-1584
- 5 Levy GG, Nichols WC, Lian EC. , et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413 (6855): 488-494
- 6 Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339 (22) 1585-1594
- 7 Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine 1966; 45 (02) 139-159
- 8 George JN. Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why?. Transfusion 2015; 55 (01) 11-13
- 9 Shah N, Rutherford C, Matevosyan K, Shen YM, Sarode R. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol 2013; 163 (04) 514-519
- 10 Li A, Makar RS, Hurwitz S. , et al. Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study. Transfusion 2016; 56 (08) 2069-2077
- 11 Bentley MJ, Lehman CM, Blaylock RC, Wilson AR, Rodgers GM. The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange. Transfusion 2010; 50 (08) 1654-1664
- 12 Bentley MJ, Wilson AR, Rodgers GM. Performance of a clinical prediction score for thrombotic thrombocytopenic purpura in an independent cohort. Vox Sang 2013; 105 (04) 313-318
- 13 Coppo P, Bengoufa D, Veyradier A. , et al; Réseau d'Etude des Microangiopathies Thrombotiques de l'Adulte. Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. Medicine (Baltimore) 2004; 83 (04) 233-244
- 14 Coppo P, Schwarzinger M, Buffet M. , et al; French Reference Center for Thrombotic Microangiopathies. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One 2010; 5 (04) e10208
- 15 Steyerberg EW, Harrell Jr FE, Borsboom GJ, Eijkemans MJ, Vergouwe Y, Habbema JD. Internal validation of predictive models: efficiency of some procedures for logistic regression analysis. J Clin Epidemiol 2001; 54 (08) 774-781
- 16
Bendapudi PK,
Hurwitz S,
Fry A.
, et al. Derivation and external validation of the PLASMIC score for rapid assessment
of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 2017;
4 (04) e157-e164
Reference Ris Wihthout Link
- 17 Koopman RJ, Mainous III AG. Evaluating multivariate risk scores for clinical decision making. Fam Med 2008; 40 (06) 412-416
- 18 Wasson JH, Sox HC, Neff RK, Goldman L. Clinical prediction rules. Applications and methodological standards. N Engl J Med 1985; 313 (13) 793-799
- 19 Williams LA, Marques MB. ; Education Committee of the Academy of Clinical Laboratory Physicians and Scientists. Pathology consultation on the diagnosis and treatment of thrombotic microangiopathies (TMAs). Am J Clin Pathol 2016; 145 (02) 158-165
- 20 Rizvi MA, Vesely SK, George JN. , et al. Complications of plasma exchange in 71 consecutive patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic-uremic syndrome. Transfusion 2000; 40 (08) 896-901
- 21 McMinn Jr JR, Thomas IA, Terrell DR, Duvall D, Vesely SK, George JN. Complications of plasma exchange in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: a study of 78 additional patients. Transfusion 2003; 43 (03) 415-416
- 22 Lo GK, Juhl D, Warkentin TE, Sigouin CS, Eichler P, Greinacher A. Evaluation of pretest clinical score (4 T's) for the diagnosis of heparin-induced thrombocytopenia in two clinical settings. J Thromb Haemost 2006; 4 (04) 759-765
- 23 Rose M, Rowe JM, Eldor A. The changing course of thrombotic thrombocytopenic purpura and modern therapy. Blood Rev 1993; 7 (02) 94-103
- 24 Wyllie BF, Garg AX, Macnab J, Rock GA, Clark WF. ; Members of the Canadian Apheresis Group. Thrombotic thrombocytopenic purpura/haemolytic uraemic syndrome: a new index predicting response to plasma exchange. Br J Haematol 2006; 132 (02) 204-209
- 25 Bobbio-Pallavicini E, Gugliotta L, Centurioni R. , et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 1997; 82 (04) 429-435
- 26 Benhamou Y, Assié C, Boelle PY. , et al; Thrombotic Microangiopathies Reference Center. Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. Haematologica 2012; 97 (08) 1181-1186
- 27 Goel R, King KE, Takemoto CM, Ness PM, Tobian AA. Prognostic risk-stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion 2016; 56 (06) 1451-1458
- 28
Bohl DD,
Russo GS,
Basques BA.
, et al. Variations in data collection methods between national databases affect
study results: a comparison of the nationwide inpatient sample and national surgical
quality improvement program databases for lumbar spine fusion procedures. J Bone Joint
Surg Am 2014; 96 (23) e193
Reference Ris Wihthout Link
- 29 Cipparone CW, Withiam-Leitch M, Kimminau KS, Fox CH, Singh R, Kahn L. Inaccuracy of ICD-9 codes for chronic kidney disease: a study from two practice-based research networks (PBRNs). J Am Board Fam Med 2015; 28 (05) 678-682
- 30 Vance GA, Niederhauser A, Chauhan SP. , et al. Does the International Classification of Disease (ICD-9) code accurately identify neonates who clinically have hypoxic-ischemic encephalopathy?. Gynecol Obstet Invest 2011; 71 (03) 202-206
- 31 O'Malley KJ, Cook KF, Price MD, Wildes KR, Hurdle JF, Ashton CM. Measuring diagnoses: ICD code accuracy. Health Serv Res 2005; 40 (5 Pt 2): 1620-1639
- 32 Benhamou Y, Baudel JL, Wynckel A. , et al. Are platelet transfusions harmful in acquired thrombotic thrombocytopenic purpura at the acute phase? Experience of the French thrombotic microangiopathies reference center. Am J Hematol 2015; 90 (06) E127-E129
- 33 Goel R, Ness PM, Takemoto CM, Krishnamurti L, King KE, Tobian AA. Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality. Blood 2015; 125 (09) 1470-1476
- 34 Brazelton J, Oster RA, McCleskey B, Fuller J, Adamski J, Marques MB. Increased troponin I is associated with fatal outcome in acquired thrombotic thrombocytopenic purpura. J Clin Apher 2016; [Epub ahead of print]
- 35 Peyvandi F, Scully M, Kremer Hovinga JA. , et al; TITAN Investigators. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med 2016; 374 (06) 511-522
- 36 Patriquin CJ, Thomas MR, Dutt T. , et al. Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura. Br J Haematol 2016; 173 (05) 779-785
- 37 Shortt J, Oh DH, Opat SS. ADAMTS13 antibody depletion by bortezomib in thrombotic thrombocytopenic purpura. N Engl J Med 2013; 368 (01) 90-92
- 38 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 (06) 1765-1772
- 39 Mori Y, Wada H, Gabazza EC. , et al. Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion 2002; 42 (05) 572-580
- 40 Vesely SK, George JN, Lämmle B. , et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003; 102 (01) 60-68
- 41 Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 2004; 103 (11) 4043-4049
- 42 Scully M, Yarranton H, Liesner R. , et al. Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features. Br J Haematol 2008; 142 (05) 819-826
- 43 Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 2010; 115 (08) 1500-1511 , quiz 1662
- 44 Cataland SR, Wu HM. How I treat: the clinical differentiation and initial treatment of adult patients with atypical hemolytic uremic syndrome. Blood 2014; 123 (16) 2478-2484