Myeloid Leukemia

PRV-1 Overexpression in PV and ET 265
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Overexpression of PRV-1 Gene in Polycythemia Rubra
Vera and Essential Thrombocythemia
Maurizio Martini, Luciana Teofili, and Luigi M. Larocca
Summary
The polycythemia rubra vera 1 gene (PRV-1), a member of the urokinase-type plasminogen
activator receptor superfamily, is overexpressed in granulocytes isolated from the peripheral
blood of patients with polycythemia vera (PV) and essential thrombocythemia (ET). PRV-1
overexpression is the first reliable molecular marker of these myeloproliferative disorders, and
its detection allows us to discriminate PV and ET from secondary erythrocytosis and thrombocytosis.
PRV-1 overexpression can be investigated by several techniques, including Northern
analysis, reverse-transcription (RT)-polymerase chain reaction (PCR), and real-time PCR.
Among these, RT-PCR is the most rapid, reliable, and feasible method for the detection of
PRV-1 overexpression in highly purified peripheral blood granulocytes.
Key Words: PRV-1; polycythemia rubra vera; essential thrombocythemia; reverse transcriptase;
PCR.
1. Introduction
Chronic myeloproliferative diseases (CMD) include chronic myelogenous
leukemia (CML), polycythemia rubra vera (PV), essential thrombocythemia
(ET), and chronic idiophatic myelofibrosis (IM) (1–5). The presence of the Ph
chromosome or its molecular equivalent, the BCR-ABL rearrangement, indubitably
indicates a diagnosis of CML. In contrast, PV and ET, the most frequent
CMD, do not exhibit a typical molecular marker, and their diagnosis
may be performed only by excluding all possible causes of secondary erythrocytosis
and thrombocytosis (6,7). Recently, Temerinac et al. cloned a novel
gene, named PRV-1, that is overexpressed in the peripheral blood granulocytes
of patients with PV and in some cases of ET (8). The PRV-1 gene codes for a
hematopoietic cell-surface receptor belonging to the superfamily of urokinasetype
plasminogen activator receptors (9). In their study, Temerinac et al. car-
From: Methods in Molecular Medicine, Vol. 125: Myeloid Leukemia: Methods and Protocols
Edited by: H. Iland, M. Hertzberrg, and P. Marlton © Humana Press Inc., Totowa, NJ
265