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Polycythemia vera and the emperor's new clothes

Source: www.haematologica.org
Topic: Polycythemia Vera
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proclivity of the incidence of polycythemia vera to increase with age. 15,16 Although, the natural history of disease manifestations appeared accelerated in some young patients, 2 this has not been a universal observation, and with the exception of an increased incidence of splenomegaly 16 as well as intra-abdominal venous thrombosis in young women, 2,18 the clinical manifestations of the disease are not different in this age group. The contention that the disease is not truly different in younger patients is bolstered not only by the rarity of the disease in childhood but also its generally milder manifestations in children as compared with any age group in adults. 19 It is also noteworthy that patients with documented familial polycythemia vera usually do not manifest the disorder clinically until later in life. 20,21 This suggests that a nite period is required for the necessary genetic changes to be acquired after the triggering insult before phenotypic expression of the disease can occur. At the same time, leaving age considerations aside, it can be argued that polycythemia vera is not a monolithic disorder. This argument is bolstered by its varied initial clinical presentations as idiopathic myelobrosis, 22 essential thrombocytosis 23 or idiopathic erythrocytosis 15 the varied tempo of disease progression in different patients, and their differing responses to treatment. Furthermore, although it was established many years ago that polycythemia vera, along with its companion myeloproliferative disorders, idiopathic myelobrosis (IMF) and essential thrombocytosis (ET), is a clonal disorder arising in a multipotent hematopoietic progenitor cell, 23 more recent and extensive clonality studies suggest that in some patients, the disorder may be polyclonal 25 and occasionally, restricted to erythroid progenitor cells. 26 Simil ...

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