Abstract
Essential thrombocythemia (ET) is a heterogeneous disorder. For example, the growth of erythropoietin-independent erythroid colonies, termed “endogenous erythroid colonies (EECs)”, has previously been observed in only 50% of ET patients. We have recently described the overexpression of a hematopoietic receptor, PRV-1 (polycythemia rubra vera-1), in patients with polycythemia vera (PV). Here, we compare PRV-1 expression and EEC formation in a cohort of 30 patients with ET; 50% of the ET patients in our cohort displayed EEC growth. Likewise, 50% of the ET patients overexpressed PRV-1. Remarkably, only the 15 ET patients displaying EEC growth showed elevated PRV-1 expression, while the 15 EEC-negative ET patients expressed normal PRV-1 levels. It has previously been reported that EEC-positive ET patients develop PV during long-term follow-up. Here, we show that 40% of the PRV-1-positive patients develop symptoms of PV during the course of their disease. In contrast, none of the 15 PRV-1-negative patients displayed such symptoms (p=0.017). Moreover, PRV-1-positive patients had a significantly higher number of thromboembolic or microcirculatory events (p=0.003). We propose that PRV-1-positive ET comprise a pathophysiologically distinct subgroup of patients, one that is at risk for the development of complications and for the emergence of PV.
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The authors sincerely appreciate Professor Dr. Geiger’s support of this work and thank H. Iwan for excellent technical assistance and C. Steimle and P. Goerttler for helpful contributions.
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This work was supported by the Alfried Kupp Förderpreis für Junge Hochschullehrer, the Else Kröner-Fresenius-Stiftung and the SFB 364, TP A12, granted to H. L. P. as well as by the German Kompetenznetz “Acute and Chronic Leukemias” (Project 25)
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Griesshammer, M., Klippel, S., Strunck, E. et al. PRV-1 mRNA expression discriminates two types of essential thrombocythemia. Ann Hematol 83, 364–370 (2004). https://doi.org/10.1007/s00277-004-0864-9
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DOI: https://doi.org/10.1007/s00277-004-0864-9