Myeloproliferative Neoplasms, Version 3.2022, NCCN Clinical Practice Guidelines in Oncology

Gerds, Aaron T.; Gotlib, Jason; Ali, Haris; Bose, Prithviraj; Dunbar, Andrew; Elshoury, Amro; George, Tracy I.; Gundabolu, Krishna; Hexner, Elizabeth O.; Hobbs, Gabriela; Jain, Tania; Kaesberg, Paul; Kuykendall, Andrew; Madanat, Yazan F.; McMahon, Brandon; Mohan, Sanjay; Nadiminti, Kalyan; Oh, Stephen T.; Pardanani, Animesh; Podoltsev, Nikolai A.; Rein, Lindsay; Salit, Rachel B.; Stein, Brady L.; Talpaz, Moshe; Vachhani, Pankit; Wadleigh, Martha; Wall, Sarah; Ward, Dawn C.; Bergman, Mary Anne; Hochstetler, Cindy L.

doi:10.6004/jnccn.2022.0046

Cited by 64 publications

(42 citation statements)

References 254 publications

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“…This aspect is interesting to harmonize the quantitation of this fibrosis in different laboratories [ 66 ]. Indeed, ET and pre-PMF do not share the same risk of progression towards myelofibrosis (at 15 years, 9% versus 17% in ET and pre-PMF, respectively) and leukemic transformation (at 15 years, 2% versus 12% in ET and pre-PMF, respectively) [ 45 , 67 ]. Rare patients (less than 5% at 10 years) display an evolute form with indirect signs of myelofibrosis on the blood smear (poikilocytosis, teardrop-shaped RBC, leukoerythroblastosis), or circulating blasts (≥20%) in the case of transformation in AL [ 68 ].…”

Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

“…Compared with other MPN, in pre-PMF, MKs are more atypical and represent a strong argument to diagnose pre-PMF and not ET [ 61 ]. This cytological distinction is very important because pre-PMF will evolve more often and/or rapidly towards overt PMF than ET [ 67 , 82 ]. Lymphoid reactional nodules are present in about a quarter of cases [ 83 ].…”

Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

“…In PMF, additional mutations play a central role in the development of prognostic scores: IPSS, DIPSS, DIPSS-Plus, MIPSS70, MIPSS70+ version 2, GIPSS and MYSEC-PM for secondary myelofibrosis to categorize patients for the risk of evolution. GIPPS is exclusively based on genetic and cytogenetic data [ 51 , 53 , 67 , 94 , 95 ]. Therefore, in clinical practice, NGS is recommended to improve risk stratification even if it is not mandatory to establish the optimal treatment [ 78 ].…”

Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

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Cytological Diagnosis of Classic Myeloproliferative Neoplasms at the Age of Molecular Biology

Combaluzier¹,

Quessada

Abbou

et al. 2023

Cells

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Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell-derived disorders characterized by uncontrolled proliferation of differentiated myeloid cells. Two main groups of MPN, BCR::ABL1-positive (Chronic Myeloid Leukemia) and BCR::ABL1-negative (Polycythemia Vera, Essential Thrombocytosis, Primary Myelofibrosis) are distinguished. For many years, cytomorphologic and histologic features were the only proof of MPN and attempted to distinguish the different entities of the subgroup BCR::ABL1-negative MPN. World Health Organization (WHO) classification of myeloid neoplasms evolves over the years and increasingly considers molecular abnormalities to prove the clonal hematopoiesis. In addition to morphological clues, the detection of JAK2, MPL and CALR mutations are considered driver events belonging to the major diagnostic criteria of BCR::ABL1-negative MPN. This highlights the preponderant place of molecular features in the MPN diagnosis. Moreover, the advent of next-generation sequencing (NGS) allowed the identification of additional somatic mutations involved in clonal hematopoiesis and playing a role in the prognosis of MPN. Nowadays, careful cytomorphology and molecular biology are inseparable and complementary to provide a specific diagnosis and to permit the best follow-up of these diseases.

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Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

Section: Bcr::abl1 -Negative Mpnmentioning

confidence: 99%

See 1 more Smart Citation

Cytological Diagnosis of Classic Myeloproliferative Neoplasms at the Age of Molecular Biology

Combaluzier¹,

Quessada

Abbou

et al. 2023

Cells

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“…Molecular analysis may, therefore, aid in treatment decisions such as allogeneic stem cell transplantation. 22,23 This review aims to highlight the role of molecular diagnostics in each of these entities categorized under BCR::ABL1-negative MPN, as well as the need for a structured diagnostic testing algorithm in aiding rapid and cost-effective diagnosis.…”

Section: Introductionmentioning

confidence: 99%

A Review on the Role of Molecular Genetics in the Diagnostic Workup of BCR::ABL1-Negative Myeloproliferative Neoplasms

Maddali

Arunachalam

Kapadia

et al. 2023

Indian J Med Paediatr Oncol

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The diagnostic evaluation of myeloproliferative neoplasms (MPNs) depends on the close correlation between clinical features, morphologic assessment of a trephine bone marrow biopsy, and molecular markers. Typically, MPNs have driver mutations in JAK2, CALR, or MPL, as well as mutations in genes related to epigenetic regulation, RNA splicing, and signaling. Mutations in these genes are a hallmark of diagnostic, prognostic, and therapeutic assessment in patients with MPNs. In line with the World Health Organization classification, all myeloproliferative disorders require molecular characterization to support diagnoses or confirm entities defined by underlying molecular abnormalities. A structured molecular analysis workflow is essential for a rapid and cost-effective diagnosis of MPN. The purpose of this review is to explore the role of molecular diagnostics in the assessment of BCR::ABL1-negative MPNs.

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“…However, with a total of at least four available TKI options, there is still a challenge when choosing the optimal first-line TKI to achieve the best therapeutic response and deep molecular response (DMR). National Comprehensive Cancer Net (NCCN) guidelines recommend using the Sokal, Hasford, EUTOS and ELTS scoring systems for CML-chronic phase (CML-CP) patients prior to the initiation of TKI therapy [ 8 , 9 , 10 , 11 , 12 ]. Although risk scores have been developed to predict the responses and/or outcomes of CML patients, few studies have critically compared them as predictors in the evaluation of molecular responses.…”

Section: Introductionmentioning

confidence: 99%

Targeted Next-Generation Sequencing Identifies Additional Mutations Other than BCR∷ABL in Chronic Myeloid Leukemia Patients: A Chinese Monocentric Retrospective Study

Chen²,

Xu³

et al. 2022

Cancers

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A proportion of patients with somatic variants show resistance or intolerance to TKI therapy, indicating additional mutations other than BCR∷ABL1 may lead to TKI treatment failure or disease progression. We retrospectively evaluated 151 CML patients receiving TKI therapy and performed next-generation sequencing (NGS) analysis of 22 CML patients at diagnosis to explore the mutation spectrum other than BCR∷ABL1 affecting the achievement of molecular responses. The most frequently mutated gene was ASXL1 (40.9%). NOTCH3 and RELN mutations were only carried by subjects failing to achieve a major molecular response (MMR) at 12 months. The distribution frequency of ASXL1 mutations was higher in the group that did not achieve MR4.0 at 36 months (p = 0.023). The achievement of MR4.5 at 12 months was adversely impacted by the presence of >2 gene mutations (p = 0.024). In the analysis of clinical characteristics, hemoglobin concentration (HB) and MMR were independent factors for deep molecular response (DMR), and initial 2GTKI therapy was better than 1GTKI in the achievement of molecular response. For the scoring system, we found the ELTS score was the best for predicting the efficacy of TKI therapy and the Socal score was the best for predicting mutations other than BCR∷ABL.

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Myeloproliferative Neoplasms, Version 3.2022, NCCN Clinical Practice Guidelines in Oncology

Cited by 64 publications

References 254 publications

Cytological Diagnosis of Classic Myeloproliferative Neoplasms at the Age of Molecular Biology

Cytological Diagnosis of Classic Myeloproliferative Neoplasms at the Age of Molecular Biology

A Review on the Role of Molecular Genetics in the Diagnostic Workup of BCR::ABL1-Negative Myeloproliferative Neoplasms

Targeted Next-Generation Sequencing Identifies Additional Mutations Other than BCR∷ABL in Chronic Myeloid Leukemia Patients: A Chinese Monocentric Retrospective Study

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