Pediatric myeloproliferative neoplasms

The increasing knowledge accumulated over recent years in adults with Philadelphia-negative myeloproliferative disorders (Ph-MPD) has prompted better evaluation of the rare pediatric essential thrombocythemia (ET), polycythemia vera (PV) and primary idiopathic myelofibrosis (PMF). A few cases of PV, ET and PMF in children were published in the English literature and an up-date of these works is here given. The experience recently collected by the Italian Pediatric Hemato-Oncology Association is also reported. Overall, the findings suggest that pediatric Ph-negative MPD are heterogeneous diseases and before the diagnosis is made, hereditary disorders have to be excluded. Because JAK2 and other genes mutations are only detectable in a minority of children with sporadic forms, complementary markers, such as clonality of hematopoieis, spontaneous erythroid colony growth, and so on should be performed for the diagnosis and new tests have to be identified. Diagnostic criteria that fit pediatric ET, PV and PMF have to be considered. Prognosis in children is still unknown and treatment guidelines need to be established; therefore, prospective observations and clinical trials are needed.