Nuclear factor-erythroid 2, nerve growth factor receptor, and CD34–microvessel density are differentially expressed in primary myelofibrosis, polycythemia vera, and essential thrombocythemia ★

SummaryBecause of the presence of various overlapping findings, the discrimination of polycythemia vera (PV) from prefibrotic/fibrotic primary myelofibrosis (PF/F-PMF) and essential thrombocythemia (ET) may be challenging, particularly in suboptimal bone marrow biopsy specimens. In this study, we assessed whether differences in the expression of nuclear factor-erythroid 2 (NF-E2), nerve growth factor receptor (NGFR; CD271), CD34, CD68, p53, CD3, CD20, and CD138 by immunohistochemistry could be useful in separating among them. Higher frequencies of nuclear positive erythroblasts with NF-E2 were observed in ET and PV cases (50% ± 13.3% and 41.5% ± 9.4%, respectively) when compared with both PF-PMF (21% ± 11.7%) and F-PMF (28.5% ± 10.8%). We found that with a cutoff level of at least 30% nuclear staining for NF-E2 in erythroblasts, we could reliably exclude the possibility of PMF. Conversely, NGFR+ stromal cells per high-power field (HPF) was significantly increased in F-PMF (53.5 ± 19.1/HPF) and PF-PMF (13.5 ± 3.8/HPF) compared with ET (4.4 ± 2.2/HPF) and PV (6.6 ± 3.3/HPF). Similarly, differences in CD34–microvessel density was remarkable in F-PMF and PF-PMF cases in comparison with PV and ET (49.9 ± 12.1/HPF, 29.3 ± 12.4/HPF, 13.7 ± 4.6/HPF, and 11.9 ± 5.1/HPF, respectively). Thus, the assessment of NF-E2 and NGFR expression and the evaluation of CD34–microvessel density may provide additional support in reaching a correct diagnosis in these cases of myeloproliferative neoplasms.