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Blood ; 122(1): 93-9, 2013 Jul 04.
Artículo en Inglés | MEDLINE | ID: mdl-23670178

RESUMEN

The World Health Organization (WHO) classification of myeloproliferative neoplasms (MPNs) comprises several entities including essential thrombocythemia (ET); primary myelofibrosis (PMF); and MPN, unclassifiable (MPN,U). Differential diagnosis between ET and early, prefibrotic PMF can be challenging but is critical because clinical course and outcome vary considerably between these entities. We have previously shown that the transcription factor nuclear factor erythroid 2 (NF-E2) is aberrantly expressed in MPN patients. Here we demonstrate that NF-E2 is mislocalized in PMF cells and that aberrant NF-E2 localization discriminates statistically highly significantly between ET and PMF. A threshold of 20% nuclear NF-E2 staining was cross-validated by ".682+ bootstrapping." Moreover, this cutoff correctly classifies diagnostic bone marrow biopsies of MPN,U patients specified upon follow-up as ET or PMF with 92% accuracy. Because interobserver concordance between independent pathologists was high (Spearman's rank correlation coefficient, 0.727), we propose that quantitative NF-E2 immunohistochemistry represents a diagnostic tool that can reliably support a differential diagnosis between ET and PMF.


Asunto(s)
Células Eritroides/metabolismo , Subunidad p45 del Factor de Transcripción NF-E2/metabolismo , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/patología , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/patología , Antígenos CD/metabolismo , Biopsia , Estudios de Cohortes , Diagnóstico Diferencial , Células Eritroides/patología , Humanos , Inmunohistoquímica/estadística & datos numéricos , Variaciones Dependientes del Observador , Pronóstico , Receptores de Transferrina/metabolismo , Bancos de Tejidos
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