Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications

The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues was last updated in 2008. Since then, there have been numerous advances in the identification of unique biomarkers associated with some myeloid neoplasms and acute leukemias, largely derived from gene expression analysis and next-generation sequencing that can significantly improve the diagnostic criteria as well as the prognostic relevance of entities currently included in the WHO classification and that also suggest new entities that should be added. Therefore, there is a clear need for a revision to the current classification. The revisions to the categories of myeloid neoplasms and acute leukemia will be published in a monograph in 2016 and reflect a consensus of opinion of hematopathologists, hematologists, oncologists, and geneticists. The 2016 edition represents a revision of the prior classification rather than an entirely new classification and attempts to incorporate new clinical, prognostic, morphologic, immunophenotypic, and genetic data that have emerged since the last edition. The major changes in the classification and their rationale are presented here.

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“…A prior well-documented diagnosis of a MPN would also generally exclude CMML or another type of MDS/MPN. 36,37 Atypical CML, BCR-ABL1…”

Section: Chronic Myelomonocytic Leukemiamentioning

confidence: 99%

The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia

Arber

Orazi

Hasserjian

et al. 2016

Blood

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“…The medical records were subsequently reviewed to obtain patients’ clinical, molecular and survival data. Consecutive 4‐μm‐thick sections were cut from the formalin‐fixed paraffin‐embedded tissue block of the skin biopsy and subjected to immunohistochemical analyses with antibodies against CD68, S100, CD1a, CD21, CD23, CD34, MPO, CD14, CD117, tryptase, and Ki67, as described previously . Positive and negative controls were included in each slide run.…”

Section: Methodsmentioning

confidence: 99%

Clonal reticulohistiocytosis of the skin and bone marrow associated with systemic mastocytosis and acute myeloid leukaemia

et al. 2017

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“…Considering instead PV patients, in a previous study involving the same cooperative group, absolute neutrophilic leukocytosis (≥13 × 10 9 L) developed at or around the time of evolution in post-polycythemic MF, was associated with a worse outcome: four patients out of 10 died after developing leukocytosis and one experienced worsening disease. In addition, when compared with a control group of post-polycythemic MF patients ( n = 23) who never developed persistent leukocytosis, the former showed a shorter OS, suggesting that persistent leukocytosis could be associated with an overall more aggressive course of the disease [39]. Interestingly, the development of leukocytosis was not associated with changes in JAK2 and BCR-ABL1 status or cytogenetic evolution.…”

Section: Morphological and Histological Characteristics Of Mpn Promentioning

confidence: 99%

Blast Transformation in Myeloproliferative Neoplasms: Risk Factors, Biological Findings, and Targeted Therapeutic Options

Iurlo

Cattaneo

Gianelli

2019

IJMS

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Myeloproliferative neoplasms represent a heterogenous group of disorders of the hematopoietic stem cell, with an intrinsic risk of evolution into acute myeloid leukemia. The frequency of leukemic evolution varies according to myeloproliferative neoplasms subtype. It is highest in primary myelofibrosis, where it is estimated to be approximately 10–20% at 10 years, following by polycythemia vera, with a risk of 2.3% at 10 years and 7.9% at 20 years. In essential thrombocythemia, however, transformation to acute myeloid leukemia is considered relatively uncommon. Different factors are associated with leukemic evolution in myeloproliferative neoplasms, but generally include advanced age, leukocytosis, exposure to myelosuppressive therapy, cytogenetic abnormalities, as well as increased number of mutations in genes associated with myeloid neoplasms. The prognosis of these patients is dismal, with a medium overall survival ranging from 2.6–7.0 months. Currently, there is no standard of care for managing the blast phase of these diseases, and no treatment to date has consistently led to prolonged survival and/or hematological remission apart from an allogeneic stem cell transplant. Nevertheless, new targeted agents are currently under development. In this review, we present the current evidence regarding risk factors, molecular characterization, and treatment options for this critical subset of myeloproliferative neoplasms patients.

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Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications

Cited by 24 publications

References 30 publications

The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia

The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia

Clonal reticulohistiocytosis of the skin and bone marrow associated with systemic mastocytosis and acute myeloid leukaemia

Blast Transformation in Myeloproliferative Neoplasms: Risk Factors, Biological Findings, and Targeted Therapeutic Options

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