Practical Implications of the 2016 Revision of the World Health Organization Classification of Lymphoid and Myeloid Neoplasms and Acute Leukemia

2017 ◽  
Vol 35 (23) ◽  
pp. 2708-2715 ◽  
Author(s):  
John P. Leonard ◽  
Peter Martin ◽  
Gail J. Roboz
Keyword(s):  
Acute Leukemia ◽  
Clinical Trial Design ◽  
Daily Practice ◽  
World Health ◽  
Myeloid Neoplasms ◽  
New Information ◽  
Health Organization ◽  
Key Aspects ◽  
Practical Implications

A major revision of the WHO classification of lymphoid and myeloid neoplasms and acute leukemia was released in 2016. A key motivation for this update was to include new information available since the 2008 version with clinical relevance for the diagnosis, prognosis, and therapy of patients. With > 100 entities described, it is important for the clinician to understand features that may be important in daily practice, whereas researchers need to incorporate the new classification scheme into study development and analysis. In this review, we highlight the key aspects of the 2016 update with particular importance to routine patient care and clinical trial design.

The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes

Blood ◽  
2009 ◽  
Vol 114 (5) ◽  
pp. 937-951 ◽  
Author(s):  
James W. Vardiman ◽  
Jüergen Thiele ◽  
Daniel A. Arber ◽  
Richard D. Brunning ◽  
Michael J. Borowitz ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Lymphoid Tissues ◽  
Myeloid Neoplasms ◽  
The World ◽  
Genetic Features ◽  
Health Organization

Recently the World Health Organization (WHO), in collaboration with the European Association for Haematopathology and the Society for Hematopathology, published a revised and updated edition of the WHO Classification of Tumors of the Hematopoietic and Lymphoid Tissues. The 4th edition of the WHO classification incorporates new information that has emerged from scientific and clinical studies in the interval since the publication of the 3rd edition in 2001, and includes new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities—some defined principally by genetic features—that have only recently been characterized. In this paper, the classification of myeloid neoplasms and acute leukemia is highlighted with the aim of familiarizing hematologists, clinical scientists, and hematopathologists not only with the major changes in the classification but also with the rationale for those changes.

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

Blood ◽  
2016 ◽  
Vol 127 (20) ◽  
pp. 2391-2405 ◽  
Author(s):  
Daniel A. Arber ◽  
Attilio Orazi ◽  
Robert Hasserjian ◽  
Jürgen Thiele ◽  
Michael J. Borowitz ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Lymphoid Tissues ◽  
Acute Leukemias ◽  
Myeloid Neoplasms ◽  
The World ◽  
Health Organization

Abstract 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.

scholarly journals How I diagnose and treat NPM1-mutated AML

Blood ◽  
2020 ◽  
Author(s):  
Brunangelo Falini ◽  
Lorenzo Brunetti ◽  
Maria Paola Martelli
Keyword(s):  
Myeloid Leukemia ◽  
Residual Disease ◽  
World Health ◽  
Gene Encoding ◽  
Multifunctional Protein ◽  
Myeloid Neoplasms ◽  
Mutational Status ◽  
Therapeutic Decisions ◽  
Health Organization

Mutations of the nucleophosmin (NPM1) gene, encoding for a nucleolar multifunctional protein, occur in about one-third of adult acute myeloid leukemia (AML). NPM1-mutated AML exhibits unique molecular, pathological and clinical features, that led to its recognition as distinct entity in the 2017 World Health Organization (WHO) classification of myeloid neoplasms. Although WHO criteria for the diagnosis of NPM1-mutated AML are well established, its distinction from other AML entities may be sometimes difficult. Moreover, the percentage of blasts required to diagnose NPM1-mutated AML remains controversial. According to the European LeukemiaNet (ELN), determining the mutational status of NPM1 (together with FLT3) is mandatory for accurate relapse risk assessment. NPM1 mutations are ideal targets for measurable residual disease (MRD) monitoring since they are AML-specific, frequent, very stable at relapse and do not drive clonal hematopoiesis of undetermined significance. MRD monitoring by quantitative PCR of NPM1 mutant transcripts, possibly combined with the ELN genetic-based risk stratification, can guide therapeutic decisions at post-remission stage. Furthermore, immunohistochemistry can be very useful in selected situations, such as diagnosis of NPM1-mutated myeloid sarcoma. Herein, we present four illustrative cases of NPM1-mutated AML, with the aim to address important issues on the biology, diagnosis and therapy of this common form of leukemia.

The World Health Organization (WHO) classification of the myeloid neoplasms

Blood ◽  
2002 ◽  
Vol 100 (7) ◽  
pp. 2292-2302 ◽  
Author(s):  
James W. Vardiman ◽  
Nancy Lee Harris ◽  
Richard D. Brunning
Keyword(s):  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Classification Schemes ◽  
Myeloid Neoplasms ◽  
Lymphoid Neoplasms ◽  
The World ◽  
Health Organization ◽  
Collaborative Efforts

A World Health Organization (WHO) classification of hematopoietic and lymphoid neoplasms has recently been published. This classification was developed through the collaborative efforts of the Society for Hematopathology, the European Association of Hematopathologists, and more than 100 clinical hematologists and scientists who are internationally recognized for their expertise in hematopoietic neoplasms. For the lymphoid neoplasms, this classification provides a refinement of the entities described in the Revised European-American Lymphoma (REAL) Classification—a system that is now used worldwide. To date, however, there has been no published explanation or rationale given for the WHO classification of the myeloid neoplasms. The purpose of this communication is to outline briefly the WHO classification of malignant myeloid diseases, to draw attention to major differences between it and antecedent classification schemes, and to provide the rationale for those differences.

scholarly journals Myeloid Neoplasm With Germline Predisposition: A 2016 Update for Pathologists

2018 ◽  
Vol 143 (1) ◽  
pp. 13-22 ◽  
Author(s):  
Juehua Gao ◽  
Shunyou Gong ◽  
Yi-Hua Chen
Keyword(s):  
Myeloid Leukemia ◽  
Short Review ◽  
World Health ◽  
World Health Organization Classification ◽  
Myeloid Neoplasm ◽  
Myeloid Neoplasms ◽  
Unique Approach ◽  
Health Organization

Context.— Myeloid neoplasms with familial occurrence have been rarely reported in the past. With the advance of molecular technology and better understanding of the molecular pathogenesis of myeloid neoplasms, investigating the genetic causes of familial acute myeloid leukemia or myelodysplastic syndrome has become feasible in the clinical setting. Recent studies have identified a rapidly expanding list of germline mutations associated with increased risks of developing myeloid neoplasm in the affected families. It is important to recognize these entities, as such a diagnosis may dictate a unique approach in clinical management and surveillance for the patients and carriers. Objective.— To raise the awareness of myeloid neoplasms arising in the setting of familial inheritance among practicing pathologists. Data Sources.— Based on recent literature and the 2016 revision of the World Health Organization classification of hematopoietic neoplasms, we provide an up-to-date review of myeloid neoplasm with germline predisposition. Conclusions.— This short review focuses on the clinical, pathologic, and molecular characterization of myeloid neoplasm with germline predisposition. We emphasize the important features that will help practicing pathologists to recognize these newly described entities.

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