Myelodysplastic syndromes (MDS) are a heterogenous group of clonal stem cell disorders which generally occur in older adults but may also affect children. Primary MDS should be distinguished from secondary MDS associated with antineoplastic or immunosuppressive therapy (t-MDS), exposure to toxic compounds, or genetic disorders. Chronic myelomonocytic leukemia (CMML) has been considered a variant of MDS and fibrotic forms have been observed in up to 16% of cases. Accordingly, the histologic evaluation of a trephine bone marrow biopsy is of critical importance for the evaluation of fibrotic or hypocellular MDS since these patterns are not reflected by the cytological examination.
On the other hand, BCR-ABL negative myeloproliferative neoplasms (MPNâs) include essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (MF), the latter notable for harboring the greatest morbidity and mortality amongst the subclass. The biological underscoring of these clonal hemopathies has historically stifled meaningful advances in gene-targeted therapies and compromised inhibition of the pro-inflammatory cytokines foundational to symptom development. The discovery of the Janus kinase 2 (JAK2) gain-of-function mutation, JAK2V617F, in 50-60% of patients with MF has led to the development of JAK2 inhibitors to treat the disease. This past decade has since been marked by the rapid development of JAK2 inhibitor therapies capable of reducing splenomegaly, cytopenias and constitutional symptoms with minimal myelosuppression and secondary toxicities.
Ruiz-ArgÃ¼elles, Ruxolitinib Chronic Myelomonocytic Leukemia-Associated Myelofibrosis: A Case Report
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Last date updated on July, 2014