The role of the Platelet Function Analyzer (PFA)-100 and platelet aggregometry in the differentiation of essential thrombocythemia from reactive thrombocytosis

Abstract

Introduction The most crucial component of all diagnostic criteria for essential thrombocythemia (ET) has been the exclusion of reactive thrombocytosis (RT). Our aim was to evaluate the diagnostic performance of the PFA-100 collagen-epinephrine (CEPI) cartridge test and epinephrine-induced aggregometry individually, but mainly combined, in the differentiation of ET from RT. Materials and Methods 26 patients with ET and 25 with RT were studied. Platelet function was analyzed by the PFA-100 and by light transmission aggregometry with epinephrine and ADP. The JAK2 mutational status was identified and hematological parameters, plasma von Willebrand factor antigen and activity levels were also assessed. Results The sensitivity (Se), specificity (Sp), positive predictive value (PPV), and the negative predictive value (NPV) of PFA-100 CEPI vs epinephrine-induced aggregometry in the differentiation of ET from RT were estimated as follows: Se (%): 78.9 vs 84.6, Sp (%): 92.0 vs 96.0, PPV (%): 88.2 vs 95.7, NPV (%): 85.2 vs 85.7, respectively. When both of these methods were combined, a lower sensitivity of 68.4%, but a specificity of 100% was attained. The PPV observed with this double abnormal combination was 100% and the NPV 80.6%. Lastly, when we assessed the abnormality for either CEPI CT or epinephrine-induced aggregometry, the sensitivity was 100%, the specificity 88.0%, PPV 86.4% and NPV 100%. Thus, an abnormal combination was strongly suggestive of ET, while normal results with both methods excluded ET. Conclusions If our results are replicated by further studies, these two methods could be used very effectively as adjunct markers in the differentiation between ET and RT.