Myeloproliferative neoplasms (MPNs) – Part 1: An overview of the diagnosis and treatment of the “classical” MPNs

Abstract

Myeloproliferative neoplasms (MPNs) are rare, yet potentially life-threatening, disorders caused by overproliferation of bone marrow stem cells. The symptom burden experienced by patients with the BCR-ABL1-negative MPNs (also referred to as the classical MPNs, i.e., essential thrombocythemia [ET], polycythemia vera [PV] and myelofibrosis [MF]) can be significant and can negatively impact quality of life (QOL). Since patients with these MPNs can live for several years, thereby requiring long-term treatment and follow-up, nurses play an essential role in communicating with these patients, assessing their symptoms, and educating them on treatments and self-management strategies that can reduce their symptom burden. This article, which is the first of a two-part series, was developed to provide nurses and other healthcare professionals with a review of the diagnosis and treatment of the most common classical MPNs. The second article in this series (also available in this issue) will provide nurses with practical guidance for managing the symptom burden associated with MPNs in order to help enhance the overall health and well-being of patients living with these disorders.

Targeting Pim kinases in hematological cancers: molecular and clinical review

“…MPNs are characterized not only by their cytology, but also the high level of JAK2 mutations. 95% of PVs and 50–60% of ETs and MFs harbor JAK2 mutations [ 132 ]. In addition, mutations in calreticulin (CALR) and MPL (thrombopoietin receptor), particularly the MPLW515L/K mutation, which signals through JAK2, are frequently seen.…”

Extramedullary Hematopoiesis of the Liver and Spleen

“…The diagnosis of MPNs is made through a series of evaluations. These include complete blood count (elevated levels of one or more cell lines), blood smear (leukoerythroblastosis and giant platelets), electrolytes, leukocyte alkaline phosphatase, uric acid, lactate dehydrogenase, erythropoietin level, von Willebrand factor determination in patients with platelets over 1 million/µL, bone marrow aspiration and biopsy (hypercellularity), cytogenetic analysis for Philadelphia chromosome, and molecular testing for JAK2, MPL, CALR, and CSF3R [72].…”

Acute Myeloid Leukemia Following Myeloproliferative Neoplasms: A Review of What We Know, What We Do Not Know, and Emerging Treatment Strategies

“…Philadelphia chromosome negative myeloproliferative neoplasms are chronic myeloid disorders which exhibit terminal myeloid expansion in the peripheral blood and are most commonly associated with mutations in the JAK/STAT pathway [ 1 ]. They include polycythemia vera (PV), essential thrombocytosis (ET), primary myelofibrosis (PMF) and pre-fibrotic myelofibrosis [ 2 , 3 ].…”