Aspirin Responsive Sticky Platelet Mediated Thrombophilia Caused by Gain of Function Mutations in the Thrombopoietin, JAK2 and MPL Genes in Hereditary and Acquired Essential Thrombocythemia

In the 1990s it was known that thrombopoietin (TPO) stimulates megakaryocyte production both in vitro and in vivo [1-3]. Size, number, and mean geometric ploidy of megakaryocytes are much more increased by TPO as compared with other cytokines with thrombopoietic activity. Evidence for a decisive role of deregulated TPO in ET comes from observations in mice overexpressing a TPO transgene where increased TPO production resulted in a fatal myeloproliferative disorder [4]. High dose exposure to TPO, lethally irradiated mice grafted with bone marrow cells infected with a retrovirus carrying the murin TPO cDNA (TPOhigh mice) developed a lethal myeloproliferative disorder of TPO induced megakaryocytic myeloproliferation (Figure 1 and 2) with reduced erythropoiesis in the spleen and bone marrow [5,6]. Transient myelofibrosis is observed in rats receiving recombinant TPO [5].

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Author: Jan Jacques Michiels

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