Progression, transformation, and unusual manifestations of myelodysplastic syndromes and myelodysplastic-myeloproliferative neoplasms: lessons learned from the XIV European Bone Marrow Working Group Course 2019

The myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are haematologically diverse stem cell malignancies sharing phenotypic features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) that display a paradoxical bone marrow phenotype hallmarked by myeloid proliferation in the context of bone marrow dysplasia and ineffective haematopoiesis. The unfolding MDS/MPN genomic landscape has revealed numerous mutations in signalling genes, such as CBL, JAK2, NRAS, KRAS, CSF3R, and others involving the spliceosome complex. These observations suggest that comutation of genes involved in dysplasia and bone marrow failure along with those of cytokine receptor signalling may, in part, explain the dual MDS/MPN phenotype. The respective MDS/MPN diseases are identified by the type of myeloid subset which predominates in the peripheral blood. Currently there are no standard treatment recommendations for most patients with MDS/MPN. To optimize efforts to improve the management and disease outcomes, it is essential to identify meaningful clinical and biologic endpoints and standardized response criteria for clinical trials.

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The implication of identifying JAK2 V617F in myeloproliferative neoplasms and myelodysplastic syndromes with bone marrow fibrosis

Journal of Hematopathology ◽

10.1007/s12308-008-0014-8 ◽

2008 ◽

Vol 1 (2) ◽

pp. 111-117 ◽

Cited By ~ 10

Author(s):

Randall J. Olsen ◽

Cherie H. Dunphy ◽

Dennis P. O’Malley ◽

Lawrence Rice ◽

April A. Ewton ◽

...

Keyword(s):

Bone Marrow ◽

Myelodysplastic Syndromes ◽

Myeloproliferative Neoplasms ◽

Jak2 V617f ◽

Bone Marrow Fibrosis ◽

Marrow Fibrosis

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Challenges in Diagnosing Myelodysplastic Syndromes in the Era of Genetic Testing: Proceedings of the 13th Workshop of the European Bone Marrow Working Group

Pathobiology ◽

10.1159/000489678 ◽

2018 ◽

Vol 86 (1) ◽

pp. 62-75

Author(s):

Konnie M. Hebeda ◽

Alexandar Tzankov ◽

Ludmila Boudova ◽

Leonie Saft ◽

Rob P. Hasserjian ◽

...

Keyword(s):

Bone Marrow ◽

Genetic Testing ◽

Myelodysplastic Syndromes ◽

Working Group

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Persistence of immunophenotypically aberrant CD34+ myeloid progenitors is frequent in bone marrow of patients with myelodysplastic syndromes and myelodysplastic/myeloproliferative neoplasms treated with hypomethylating agents

Journal of Clinical Pathology ◽

10.1136/jclinpath-2016-203715 ◽

2016 ◽

Vol 69 (11) ◽

pp. 1001-1008 ◽

Cited By ~ 5

Author(s):

Lanshan Huang ◽

Guillermo Garcia-Manero ◽

Elias Jabbour ◽

Maitrayee Goswami ◽

Mark J Routbort ◽

...

Keyword(s):

Bone Marrow ◽

Myelodysplastic Syndromes ◽

Myeloproliferative Neoplasms ◽

Hypomethylating Agents ◽

Myeloid Progenitors

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C016 Expression analysis of proteins involved in the Non Homologous End Joining DNA repair mechanism, in the bone marrow of adult de novo myelodysplastic syndromes

Leukemia Research ◽

10.1016/s0145-2126(09)70054-2 ◽

2009 ◽

Vol 33 ◽

pp. S40-S41

Author(s):

P. Economopoulou ◽

V. Pappa ◽

F. Kontsioti ◽

S. Papageorgiou ◽

E. Liakata ◽

...

Keyword(s):

Bone Marrow ◽

Dna Repair ◽

Expression Analysis ◽

Myelodysplastic Syndromes ◽

De Novo ◽

Repair Mechanism ◽

End Joining ◽

Non Homologous End Joining

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A review of current knowledge of myeloproliferative disorders in the horse

Acta Veterinaria Scandinavica ◽

10.1186/s13028-021-00573-3 ◽

2021 ◽

Vol 63 (1) ◽

Author(s):

Katy Satué ◽

Juan Carlos Gardon ◽

Ana Muñoz

Keyword(s):

Bone Marrow ◽

Myeloid Leukemia ◽

Current Knowledge ◽

Myeloproliferative Neoplasms ◽

Myeloproliferative Disorders ◽

World Health ◽

Chronic Granulocytic Leukemia ◽

Current State ◽

Multiple Cell ◽

Health Organization

AbstractMyeloid disorders are conditions being characterized by abnormal proliferation and development of myeloid lineage including granulocytes (neutrophils, eosinophils and basophils), monocytes, erythroids, and megakaryocytes precursor cells. Myeloid leukemia, based on clinical presentation and proliferative rate of neoplastic cells, is divided into acute (AML) and myeloproliferative neoplasms (MPN). The most commonly myeloid leukemia reported in horses are AML-M4 (myelomonocytic) and AML-M5 (monocytic). Isolated cases of AML-M6B (acute erythroid leukemia), and chronic granulocytic leukemia have also been reported. Additionally, bone marrow disorders with dysplastic alterations and ineffective hematopoiesis affecting single or multiple cell lineages or myelodysplastic diseases (MDS), have also been reported in horses. MDSs have increased myeloblasts numbers in blood or bone marrow, although less than 20%, which is the minimum level required for diagnosis of AML. This review performed a detailed description of the current state of knowlegde of the myeloproliferative disorders in horses following the criteria established by the World Health Organization.

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Can Novel Insights into the Pathogenesis of Myeloproliferative Neoplasm-Related Thrombosis Inform Novel Treatment Approaches?

Hemato ◽

10.3390/hemato2020018 ◽

2021 ◽

Vol 2 (2) ◽

pp. 305-328

Author(s):

Ofir Wolach ◽

Adi Shacham Abulafia

Keyword(s):

Venous Thrombosis ◽

Myeloproliferative Neoplasms ◽

Myeloproliferative Neoplasm ◽

Lessons Learned ◽

Therapeutic Approaches ◽

Current Management ◽

Extrinsic Factors ◽

Inflammatory Conditions ◽

Novel Treatment ◽

Management Issues

Despite recent advances in diagnosis and therapy, arterial and venous thrombosis remain a major cause of morbidity and mortality in Philadelphia-negative myeloproliferative neoplasms (MPNs). Preventing and treating arterial and venous thrombosis represent one of the major goals in MPNs. The prothrombotic phenotype of MPNs is the result of a complex interplay between several components. Neutrophils, platelets, red blood cells (RBCs) and endothelial cells assume an activated phenotype in MPNs and undergo morphologic and metabolic changes that render these cells prothrombotic. These changes are in part the result of alterations induced by MPN initiating, driving mutations as well as the effect of extrinsic factors that stem from cell interactions as well as the inflammatory environment and rheological properties that characterize MPNs. In this review, we address current management issues in MPNs and provide an update on recent understanding of the pathogenesis of thrombosis in MPNs. We also address how lessons learned from other thrombo-inflammatory conditions can further inform and improve management of thrombosis in MPNs. Based on the above data and recent discoveries and developments, we discuss potential novel targets and therapeutic approaches to tackle the challenge of thrombosis in MPNs.

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