Literature review: An Introduction to Myeloproliferative Neoplasms
Michelle Butina, Nicola Richards, Nichola H Lawrence, Indu Singh
Int. J. Bio. Lab. Sci 2021(10)2:86-90【PDF】
The Philadelphia chromosome negative myeloproliferative neoplasms (MPNs) are a rare group of chronic hematological diseases that are closely related. They arise from one of three disease-initiating driver mutations that cause over-activation of the JAK-STAT pathway resulting in disease of the hematopoietic system. These three MPNs have overlapping clinical and diagnostic features making diagnosis challenging. The World Health Organization (WHO) diagnostic criteria continues to evolve as more advances are made in understanding these complex diseases. As such, prognostic models for risk stratification are also evolving and newer models (e.g., Mutation-Enhanced International Prognostic Score System) incorporate genetic and molecular features. The major and most common complications include thrombohemorrhagic manifestations and progression to acute leukemia. The development of Janus kinase inhibitors has changed the treatment landscape of MPNs, yet treatment options are at this time still limited due to the complexities of these diseases.
Key words: Myeloproliferative neoplasm, polycythemia vera, essential thrombocythemia, primary myelofibrosis.