[Myeloproliferative neoplasms - Update on diagnosis and treatment].

Meyer, Sara C.; Drexler, Beatrice; Skoda, Radek C. (2019). [Myeloproliferative neoplasms - Update on diagnosis and treatment]. Therapeutische Umschau. Revue therapeutique, 76(9), pp. 487-495. Hogrefe 10.1024/0040-5930/a001128

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Myeloproliferative neoplasms - Update on diagnosis and treatment Abstract. Myeloproliferative neoplasms are hematopoietic stem cell disorders presenting as chronic leukemias with excessive production of mature, myeloid blood cells. Driver mutations in JAK2, CALR or MPL mediate constitutive activation of JAK2 signaling, a common hallmark of the classical Philadelphia chromosome-negative MPN including polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). Dysregulated hematopoiesis primarily presents with polyglobulia in PV, thrombocytosis in ET and progressive bone marrow fibrosis and increased, atypical megakaryocytes in PMF. The molecular characterization of MPNs has advanced our understanding of their pathogenesis and has facilitated diagnosis. Implementation of genetic markers enables improved prognostication, particularly in myelofibrosis. In recent years, we have seen an encouraging increase in therapeutic options for MPN including the approval of a first JAK inhibitor now followed by other agents of this class, as well as refined forms of interferon alpha. Combination therapies as well as novel therapeutic approaches are increasingly studied and hold promise for the future. Hematopoietic stem cell transplantation, which is still the only treatment option with a curative potential, is increasingly available also for elderly patients with MPN.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory

UniBE Contributor:

Meyer, Sara Christina

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0040-5930

Publisher:

Hogrefe

Language:

German

Submitter:

Julia Elisa Garcia

Date Deposited:

22 Dec 2023 06:14

Last Modified:

22 Dec 2023 06:14

Publisher DOI:

10.1024/0040-5930/a001128

PubMed ID:

32157965

URI:

https://boris.unibe.ch/id/eprint/190296

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