2013, Number 2
<< Back Next >>
Rev Hematol Mex 2013; 14 (2)
Usefulness of PFA-100 in a Colombian Population, as a Screening Method for Von Willebrand Disease and Function Platelet Disorders
Lombana MA, Ramos-Ramos G, Milena TA
Language: Spanish
References: 44
Page: 71-77
PDF size: 362.85 Kb.
ABSTRACT
Background: The evaluation of inherited disorders of platelet function in clinical practice is hampered by the use of screening methods with poor reproducibility and operational performance. Recently the use of evidence as the PFA-100 seems to be a more appropriate method. In Colombia there is not quantified or published experience in this regard.
Objective: The main objective of this study is to present the first Colombian experience of the diagnostic performance of the PFA- 100 in patients with suspected Von Willebrand disease (VWD) and non-pharmacological functional platelet disorders.
Materials and methods: We performed a cross-sectional study of validation of diagnostic tests, with prospective data collection. We included patients with suspected VWD, functional platelet disorders and a group of healthy controls.
All patients underwent PFA-100 test independently, with simultaneous determination of Von Willebrand antigen, ristocetin cofactor, factor VIII and platelet aggregation. We determined the sensitivity, specificity, LR + and LR-of PFA-100 and bleeding time as an alternative method.
Results: 38 subjects were included from Bogota. The PFA-100 had a sensitivity of 90%, negative LR 0,12, a specificity of 85.7% and positive LR 6,2. The bleeding time had a sensitivity of 40% and negative LR 0,6.
Conclusions: In patients with suspected VWD or platelet function disorders using PFA-100 in our study group had a high diagnostic yield. It is recommended the use of PFA-100 as a screening method in patients with clinical suspicion of VWD or platelet function defects, not with bleeding time due to its poor sensitivity.
REFERENCES
Kaushansky K, Lichtman M. Von Willebrand Disease. Williams Hematology. 8th ed. 2010; 127.
Friedman K. Inherited coagulation disorders. Greer et al. Wintrobe´s Clinical Hematology. 12th ed. 2009; 57.
National Institutes of Health, National Heart, Lung, and Blood Institute (NHLBI). The diagnosis, evaluation, and management of von Willebrand disease. Bethesda (MD): U.S. Department of Health and Human Services; 2007;112.
Sadler JE. Von Willebrand Disease. Colman, Robert W. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 5th ed. 2006;60.
Kaushansky K, Lichtman M. Hereditary qualitative platelet disorders. Williams Hematology. 8th ed. 2010;121.
Kunicki TJ. Qualitative disorders of platelet function. Greer et al. Wintrobe´s Clinical Hematology. 12th ed. 2009;56.
Lind SE. The bleeding time. Platelets 2nd ed. 2007;485-493.
Kundu S, Heilmann E, Sio R, Garcia C, Davidson R, Ostgaard R. Description of an in vitro platelet function analyzer --PFA- 100. Sem Thromb Hemost 1995;21(suppl.2):106-112.
Favaloro EJ. Clinical utility of the PFA-100. Semin Thromb Hemost 2008;34:709-33. Epub 2009 Feb 12.
Quiroga T, Goycoolea M, Muñoz B, et al. Template bleeding time and PFA-100 have low sensitivity to screen patients with hereditary mucocutaneous hemorrhages: comparative study in 148 patients. Thromb Haemost 2004;2:892-898.
Dade ® PFA-100® Reagents. B4170 G20A U5735 (445) H/CS/R Edition February 2008
Kundu S, Heilmann E, Sio R, Garcia C, Ostgaard R. Characterization of an in vitro platelet function analyzer -PFA-100. Clin App Thrombosis-Hemostasis 1996;2:241-249.
Harrison P. Robinson MS, Mackie IJ, Joseph J, McDonald SJ, Liesner R, Savidge GF, Pasi J, Machin SJ. Performance of the platelet function analyzer PFA-100 in testing abnormalities of primary haemostasis. Blood Coagul Fibrinolysis 1999;10:25- 31.
Cattaneo M, Lecchi A, Agati B, Lombardi R, Zighetti M. Evaluation of platelet function with the PFA-100 system in patients with congenital defects of platelet secretion. Thromb Res 1999; 96:213-217.
Francis J, Francis D, Larson L, Helms E, Garcia M. Can the Platelet Function Analyzer (PFA®)-100 test substitute for the template bleeding time in routine clinical practice? Platelets 1999;10:132-136.
Ortel T, James A, Thames E, Moore K, Greenberg C. Assessment of primary hemostasis by PFA-100® analysis in a tertiary care center. Thromb Haemost 2000;84: 93-97.
Kretschmer V, Weippert-Kretschmer M: Determination and treatment of disorders of primary hemostasis: Experience with routine application of the in vitro bleeding test. Hämostaseologie 1999;19:168-175.
Kerenyi A, Schlammadinger A, Ajzner E, Szegedi I, Kiss C, Pap Z, Boda Z, Muszbek L. Comparison of the PFA-100 closure time and template bleeding time of patients with inherited disorders causing defective platelet function. Thromb Res1999;96:487-492.
Fressinaud E, Veyradier A, Truchaud F, Martin I, Boyer-Neumann C, Trossaert M, Meyer D. Screening for von Willebrand Disease with a new analyzer using high shear stress: A study of 60 cases. Blood 1998;91:1325-1331.
Cattaneo M, Federici AB, Lecchi A, Agati B, Lombardi R, Stabile F, Bucciarelli P. Evaluation of the PFA-100® system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease. Thromb Haemost 1999;82:35-39.
Fressinaud E, Veyradier A, Sigaud M, Boyer-Neumann C, Le Boterff C, Meyer D. Therapeutic monitoring of von Willebrand disease: interest and limits of a platelet function analyzer at high shear rates. Br J Haematol 1999;106:777-783.
Favaloro EJ, Facey D, Henniker A. Use of a novel platelet function analyzer (PFA-100) with high sensitivity to disturbances in von Willebrand factor to screen for von Willebrand’s disease and other disorders. Am J Hematol 1999;62:165-174.
Meskal A, Vertessen F, Van der Planken M, Berneman ZN. The platelet function analyzer (PFA-100) may not be suitable for monitoring the therapeutic efficiency of von Willebrand concentrate in type III von Willebrand disease. Ann Hematol 1999;78:426-430.
Kerenyi A, Schlammadinger A, Ajzner E, et al. Comparison of PFA-100 closure time and template bleeding time of patients with inherited disorders causing defective platelet function. Thromb Res 1999;96:487-492.
Harrison P, Robinson MS, Mackie IJ, et al. Performance of the platelet function analyser PFA-100 in testing abnormalities of primary haemostasis. Blood Coagul Fibrinolysis 1999;10:25-31.
Dean JA, Blanchette VS, Carcao MD, et al. von Willebrand disease in a paediatric-based population-comparison of type 1 diagnostic criteria and use of the PFA-1001 and a von Willebrand factor/collagen-binding assay. Thromb Haemost 2000;84:401-409.
Schlammadinger A, Kerenyi A, Muszbek L, Boda Z. Comparison of the O’Brien filter test and the PFA-100 platelet analyser in the laboratory diagnosis of von Willebrand´s disease. Thromb Haemost 2000;84:88-92.
Ortel TL, James AH, Thames EH, Moore KD, Greenberg CS. Assessment of primary hemostasis by PFA-100 analysis in a tertiary care center. Thromb Haemost 2000;84:93-97.
Favaloro EJ, Kershaw G, Bukuya M, Hertzberg M, Koutts J. Laboratory diagnosis of von Willebrand disorder (VWD) and monitoring of DDAVP therapy: efficacy of the PFA-100 and VWF: CBA as combined diagnostic strategies. Haemophilia 2001;7:180-189.
Harrison P, Robinson M, Liesner R, et al. The PFA-100: a potential rapid screening tool for the assessment of platelet dysfunction. Clin Lab Haematol 2002;24:225-232.
Buyukasik Y, Karakus S, Goker H, et al. Rational use of the PFA-100 device for screening of platelet function disorders and von Willebrand disease. Blood Coagul Fibrinolysis 2002;13:349-353.
Wuillemin WA, Gasser KM, Zeerleder SS, Lammle B. Evaluation of a Platelet Function Analyser (PFA-100) in patients with a bleeding tendency. Swiss Med Wkly 2002;132:443-448.
Nitu-Whalley IC, Lee CA, Brown SA, Riddell A. The role of the platelet function analyzer (PFA-100) in the characterization of patients with von Willebrand’s disease and its relationships with von Willebrand factor and the ABO blood group. Haemophilia 2003;9:298-302.
Cariappa R, Wilhite TR, Parvin CA, Luchtman-Jones L. Comparison of PFA-100 and bleeding time testing in pediatric patients with suspected hemorrhagic problems. J Pediatr Hematol Oncol 2003;25:474-479.
Posan E, McBane RD, Grill DE, Motsko CL, Nichols WL. Comparison of PFA-100 testing and bleeding time for detecting platelet hypofunction and von Willebrand disease in clinical practice. Thromb Haemost 2003;90:483-490.
Koscielny J, Ziemer S, Radtke H, et al. A practical concept for preoperative identification of patients with impaired primary hemostasis. Clin Appl Thromb Hemost 2004;10:195-204.
Philipp CS, Miller CH, Faiz A, et al. Screening women with menorrhagia for underlying bleeding disorders: the utility of the platelet function analyzer and bleeding time. Haemophilia 2005;11:497-503.
Penas N, Perez-Rodrıguez A, Torea JH, et al. Willebrand disease R1374C: type 2A or 2M? A challenge to the revised classification. High frequency in the northwest of Spain (Galicia) Am J Hematol 2005;80:188-196.
Favaloro EJ, Lloyd J, Rowell J, et al. Comparison of the pharmacokinetics of two von Willebrand factor concentrates [Biostate and AHF (High Purity)] in people with von Willebrand disorder. A randomized cross-over, multi-centre study. Thromb Haemost 2007;97:922-930.
Podda GM, Bucciarelli P, Lussana F, Lecchi A, Cattaneo M. Usefulness of PFA-100 testing in the diagnostic screening of patients with suspected abnormalities of hemostasis: comparison with the bleeding time. J Thromb Haemost 2007;5:2393-2398.
Fressinaud E, Veyradier A, Truchaud F, et al. Screening for von Willebrand disease with a new analyzer using high shear stress: a study of 60 cases. Blood 1998;91:1325-1331.
Mammen EF, Comp PC, Gosselin R, et al. PFA-100 system: a new method for assessment of platelet dysfunction. Semin Thromb Hemost 1998;24:195-202.
Tsantes AE, Dimoula A, Bonovas S, Mantzios G. The role of the Platelet Function Analyzer (PFA)-100 and platelet aggregometry in the differentiation of essential thrombocythemia from reactive thrombocytosis. Thromb Res 2010;125:142-146.
Cesar JM, de Miguel D, Garcia Avello A, Burgaleta C. Platelet dysfunction in primary thrombocythemia using the platelet function analyzer, PFA-100. Am J Clin Pathol 2005;123:772-777.