New knowledge of Philadelphia Negative Classical Myeloproliferative Neoplasms
نویسندگان
چکیده
This article tries to answer the following questions: why we are in need of a revision of the prior diagnostic criteria for Philadelphia -ve MPNs, what is new in 2016 multiparameter WHO classification for Philadelphia-ve MPNs and how a single JAK2V617F mutation can lead to three different MPNs. In addition it discusses the genetic basis and molecular pathophysiology of Philadelphia-ve classical myeloproliferative neoplasms. Abbreviations: BM: Bone Marrow; CALR: Calreticulin; ET: Essential Thrombocythemia; JAK2: Janus kinase 2; MF: Myelo-Fibrosis; MPL: MyeloProliferative Leukemia virus oncogene; MPN: Myelo-Proliferative Neoplasm; PMF: Primary Myelo-Fibrosis; PV: Polycythemia Vera; WHO: World Health Organization; Hb: Hemoglobin; VWF: Von Willebrand Factor; AVWS: Acquired Von Willebrand Syndrome; HSC: Hemopoietic Stem Cell
منابع مشابه
Low Frequency of C-MPL Gene Mutations in Iranian Patients with Philadelphia-Negative Myeloproliferative Disorders
Background Myeloproliferative disorders are a group of diseases characterized by increased proliferation of myeloid lineage. In addition to JAK2V617F mutation, several mutations in the c-MPL gene were described in patients with Philadelphia-negative chronic myeloproliferative disorders that could be important in the pathogenesis of diseases. The aim of present study was to investigate the fre...
متن کاملRecommendations for molecular testing in classical Ph1-neg myeloproliferative disorders-A consensus project of the Italian Society of Hematology.
The discovery that Philadelphia-negative classical myeloproliferative neoplasms (MPNs) present with several molecular abnormalities, including the mostly represented JAK2V617F mutation, opened new horizons in the diagnosis, prognosis, and monitoring of these disorders. However, the great strides in the knowledge on molecular genetics need parallel progresses on the best approach to methods for ...
متن کاملA Retrospective Haematological Study of Myeloproliferative Neoplasms in a Tertiary Care Centre
According to the World Health Organization (WHO) 2008 criteria, MPNs are now divided in classical MPNs which carry the Philadelphia (Ph+) chromosome (chronic myeloid leukaemia) and classical MPNs which do not carry the Philadelphia (Ph-) chromosome, including essential thrombocythemia, polycythemia vera, primary myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia, mastoc...
متن کاملRecent advances in diagnosis and treatment of chronic myeloproliferative neoplasms
The Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs) have recently been the focus of tremendous advances in basic knowledge of disease pathophysiology following the recognition of mutations in JAK2 and MPL. These discoveries also led to refinement of the criteria employed for diagnosis. The prognostic roles of the JAK2 V617F mutation and of leukocytosis as independen...
متن کامل