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Leukemias
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
The 2016 WHO revised classification recognizes myeloproliferative neoplasms (MPNs) to comprise seven subtypes: chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia, not otherwise specified (CEL), and myeloproliferative neoplasm, unclassifiable (MPN-U). In addition, the WHO 2016 classification recognizes myeloid/lymphoid neoplasms with eosinophilia and rearrangements of PDGFRA, PDGFRB, FGFR1, or PCM-JAK2 (a provisional entity), and mastocytosis (see Table 28.2). CML, PV, ET, and PMF are often referred to collectively as “classic” MPNs and includes patients who transform to myelofibrosis (MF) from PV (post-PV MF) or ET (post-ET MF); in contrast, CNL, CEL, and MPN-U are referred to as “non-classic” or atypical MPNs. The classic MPNs are in turn often divided into BCR-ABL1-positive (CML) and -negative (PV, ET, and PMF).
Acute Myeloid Leukemia
Published in Dongyou Liu, Tumors and Cancers, 2017
Myeloid neoplasms are classified into eight major entities: (i) myeloproliferative neoplasms; (ii) mastocytosis; (iii) myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1 or with PCM1-JAK2; (iv) Myelodisplastic/myeloproliferative neoplasms; (v) Myelodisplastic syndromes/neoplasms; (vi) myeloid neoplasms with germline predisposition; (vii) acute myeloid leukemia and related precursor neoplasms; and (viii) acute leukemias of ambiguous lineage [1–3].
Mesenteric vein thrombosis
Published in Peter Gloviczki, Michael C. Dalsing, Bo Eklöf, Fedor Lurie, Thomas W. Wakefield, Monika L. Gloviczki, Handbook of Venous and Lymphatic Disorders, 2017
Waldemar E. Wysokinski, Robert D. McBane
Myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are found in about a third of MVT cases and therefore are important considerations in the search for an underlying mechanism.19 These disorders represent a stem cell-derived clonal myeloproliferation. The most common clinical manifestation of this malignancy and the cause of death is venous or arterial thrombosis.20 The JAK2V617F sequence variation with gain of function that leads to independent proliferation is found in 90% of cases of polycythemia vera and up to 50% of cases of essential thrombocythemia. Screening for this mutation is therefore appropriate in the initial evaluation of patients who are suspected of having these disorders, including patients with MVT.21–23 In fact, detection of the JAK2 sequence variation has replaced bone marrow examination as the first test to screen for myeloproliferative neoplasms.22,24
Frequency of JAK2V617F, MPL and CALR driver mutations and associated clinical characteristics in a Norwegian patient cohort with myeloproliferative neoplasms
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2023
Susanne Lilleskare, Marta Vorland, Anh Khoi Vo, Aasne K. Aarsand, Håkon Reikvam
Myeloproliferative neoplasms are malignant hematological diseases characterized by abnormal growth in one or more myeloid cell lines. The disease group includes polycythemia vera, essential thrombocytosis, primary myelofibrosis and chronic myelogenous leukemia [1,2]. The latter differs from the other conditions by the presence of the Philadelphia chromosome, which gives rise to the fusion gene BCR-ABL1 [1]. Thus, further mention of myeloproliferative neoplasms relates to the three Philadelphia chromosome-negative conditions; polycythemia vera, essential thrombocytosis and primary myelofibrosis. These diseases are also characterized by an increased risk of thromboembolic events, and transformation to more malignant conditions, including acute myelogenous leukemia or myelofibrosis [2].
Updated recommendations on the use of ruxolitinib for the treatment of myelofibrosis
Published in Hematology, 2022
Timothy Devos, Dominik Selleslag, Nikki Granacher, Violaine Havelange, Fleur Samantha Benghiat
Myeloproliferative neoplasms are rare bone marrow disorders characterized by clonal proliferation of hematopoietic cell lineages, which comprise polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF) [1]. MF has worse prognosis, with main causes of death including acute leukemia transformation, comorbid conditions, and consequences of cytopenia [1,2]. MF is characterized by progressive anemia, bone marrow fibrosis, and extramedullary hematopoiesis with splenomegaly, and is associated with a heavy symptom burden (e.g. night sweats, fever, bone pain, and weight loss) [2–4]. Patients can be diagnosed with primary MF, or patients with PV or ET can develop post-PV or post-ET MF [2]. MF is associated with a dysregulation of the Janus kinase (JAK)/signal transducer and activator of transcription (STAT) pathway [5]. One of the three driver mutations (Janus kinase 2 [JAK2], calreticulin [CALR], or myeloproliferative leukemia virus oncogene [MPL]) is found in around 90% of MF patients [2,6]. The remaining ‘triple negative’ MF patients have poor clinical outcomes [6]. Other high-molecular risk (HMR) mutations contributing to disease progression include ASXL1, SRSF2, EZH2, IDH1, IDH2, and U2AF1 [2].
Fedratinib: a pharmacotherapeutic option for JAK-inhibitor naïve and exposed patients with myelofibrosis
Published in Expert Opinion on Pharmacotherapy, 2022
The myeloproliferative neoplasms are a group of clonal disorders of hematopoiesis that include essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). Myelofibrosis may exist as its own entity (primary MF), or following a preceding history of PV, or ET (secondary MF). Current National Comprehensive Cancer Network (NCCN) and European LeukemiaNet (ELN) management guidelines recommend clinical and molecular risk stratification, and consideration for curative hematopoietic stem cell transplantation (HCT) in fit patients with predicted survival ≤5 years [1–4]. Medical therapy with JAK-inhibitors (JAKi) is recommended for patients with symptomatic splenomegaly or cytokine-mediated-constitutional symptoms including fever, night-sweats, and weight loss [5–7]; with ongoing controversy as to whether JAKi-therapy improves survival in patients with symptomatic MF [8,9].