PRV-1 mRNA expression discriminates two types of essential thrombocythemia.
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.
Griesshammer
M
M
Department of Medicine III, Hematology, Oncology, Rheumatology and Infectious Diseases, University of Ulm, Robert-Koch-Strasse 5, 89081 Ulm, Germany.
Klippel
S
S
Strunck
E
E
Temerinac
S
S
Mohr
U
U
Heimpel
H
H
Pahl
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HL
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015152:Blotting, Northern; D015331:Cohort Studies; D003937:Diagnosis, Differential; D015672:Erythroid Precursor Cells / Q000166:cytology / Q000473:pathology; D005260:Female; D058851:GPI-Linked Proteins; D015870:Gene Expression; D006801:Humans; D007519:Isoantigens / Q000096:biosynthesis* / Q000235:genetics; D008297:Male; D008562:Membrane Glycoproteins / Q000096:biosynthesis* / Q000235:genetics; D008875:Middle Aged; D011087:Polycythemia Vera / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012333:RNA, Messenger / Q000096:biosynthesis* / Q000235:genetics; D011956:Receptors, Cell Surface; D012189:Retrospective Studies; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016019:Survival Analysis; D013920:Thrombocythemia, Essential / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000473:pathology
C407242:CD177 protein, human; D058851:GPI-Linked Proteins; D007519:Isoantigens; D008562:Membrane Glycoproteins; D012333:RNA, Messenger; D011956:Receptors, Cell Surface