ABSTRACT
The focus of this chapter is to summarize the history of the main biological and clinical developments achieved in the three most common Ph-negative MPD.
The history of PV began in 1892 with the initial description by Vaquez of a single patient who showed a particular form of cyanosis associated with persistent, autonomous, and extreme erythrocytosis. 1 A decade after this description, Osler delineated PV as a new clinical entity characterized by ‘cyanosis with polycythemia and enlarged spleen with symptoms somewhat indefinite and with pathology quite obscure ’.2 These clinicians thought PV was marked only by the increase of red cell count not secondary to congenital heart disease and failed to recognize that the process resulted from a global bone marrow proliferation, as evidenced by concomitant hyperplasia of granulocytic and megakaryocytic cell lines. As a consequence, erythrocytosis was considered the most prominent cause of clinical manifestations and, accordingly, much of the research regarding the pathogenesis of PV focused on erythropoiesis.
