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Diagnostic Approach to Myeloid Neoplasms Myeloid Neoplasms With Hyperproliferative Activity: MPN, Myeloid-Lymphoid Neoplasms With Eosinophilia

Diagnostic Approach to Myeloid Neoplasms Myeloid Neoplasms With Hyperproliferative Activity: MPN, Myeloid-Lymphoid Neoplasms With Eosinophilia
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Copyright: 2024
Pages: 55
Source title: Principles and Approaches to Diagnostic Bone Marrow Examination
Source Author(s)/Editor(s): Samia Hassan Rizk (Cairo University, Egypt)
DOI: 10.4018/978-1-6684-5818-1.ch010

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Abstract

Myeloproliferative neoplasms comprise BCR-ABL1 positive CML, Classic MPNs BCR-ABL1 negative, and a group of nonclassical MPNs. They share the common feature of hyperproliferation of one or more hemopoietic lineages, initially effective and gradually becoming ineffective with progression into fibrotic or leukemic phases. The fusion oncogene BCR-ABL1 is the genetic hallmark of CML. The pathogenesis of classic BCR-ABL1 negative MPNs is dynamic, with a complex interaction of tumor subclones with the bone marrow microenvironment and feedback drivers. The primary driver mutations of MPNs are the cytokine signaling pathway genes JAK2 and MPL and the loss-of-function CALR mutations, mainly through the JAK-STAT pathway. Nonclassical Philadelphia-negative MPNs: Chronic Neutrophilic Leukemia (CNL), Chronic Eosinophilic Leukemia (CEL), CEL-NOS, Juvenile MyeloMonocytic Leukemia (JMML), and MPN-unclassifiable (MPN-U), each has characteristic molecular pathogenesis.

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