These findings suggest that monitoring JAK2 (V617F) allele burden could be useful to identify patients at higher risk of
myelofibrotic transformation.
These findings were observed in multiple paediatric idiopathic
myelofibrotic patients in an extensive study by Delario and Sheehan comprising 19 children followed over a period of 30 years.5 The findings of hypercellularity (95%) and megakaryocyte hyperplasia can also be seen in the index case.
In the classical Philadelphia chromosome-negative myeloproliferative neoplasms, several lines of evidence link ASXL1 mutations with BM fibrosis: ASXL1 mutations are associated with a higher degree of BM fibrosis in primary myelofibrosis [31]; ASXL1 mutations are associated with an increased risk of
myelofibrotic transformation in patients with essential thrombocythemia and polycythemia vera [32]; and ASXL1 mutations are more frequently detected in overt as opposed to prefibrotic primary myelofibrosis [33].
Myelofibrotic variant of myelodysplastic syndrome###0 ( 0% )###1 (1.6%)###0.491
DISCUSSION: Anaemia is the most common haematological disorder caused by TB but other abnormalities occur such as leucopenia, leukaemoid reaction,
myelofibrotic changes, the haemophagocytic syndrome, polycythemia, thrombocytosis, thrombocytopenia, monocytosis.
We have seen no greater incidence of the
myelofibrotic state than in patients with polycythemia treated by other measures.