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Atypical CML-the Role of Morphology and Precision Genomics
Publication date: Available online 6 December 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Terra LashoAbstractAtypical chronic myeloid leukemia is an esoteric myeloid malignancy with features of both myeloproliferative and myelodysplastic syndromes. This disease is characterized primarily by morphologic-based criteria, and has clinical and molecular features overlapping with other myeloid malignancies. No one molecular abnormality is specific, and multiple mutations are often present in various combinations, due to the malignant multi-step clonal evolution of myeloid malignancies. In this review, we ...
Source: Best Practice and Research Clinical Haematology - December 7, 2019 Category: Hematology Source Type: research

Myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS/MPN-U): More than just a “catch-all” term?
Publication date: Available online 6 December 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Rory M. Shallis, Amer M. ZeidanAbstractThe clinicopathology of MDS and MPN are not mutually exclusive and for this reason the category of myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) exists. Several sub-entities have been included under the MDS/MPN umbrella, including MDS/MPN-unclassifiable (MDS/MPN-U) for those cases whose morphologic and clinical phenotype do not meet criteria to be classified as any other MDS/MPN sub-entity. Though potentially regarded as a wastebasket diagnosis, since its ...
Source: Best Practice and Research Clinical Haematology - December 7, 2019 Category: Hematology Source Type: research

Myelodysplastic/Myeloproliferative neoplasms – justified inclusion as unique biological entities
Publication date: Available online 7 December 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Mrinal M. Patnaik (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - December 7, 2019 Category: Hematology Source Type: research

Incorporating flow cytometry and next-generation sequencing in the diagnosis of CMML. Are we ready for prime?
Publication date: Available online 7 December 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Eric Solary, Orianne Wagner-Ballon, Dorothée Selimoglu-BuetAbstractIn the last version of the WHO classification of myeloid malignancies, flow cytometry and molecular investigation are listed as potentially useful, yet non-essential diagnostic tools in hard-to-recognize chronic myelomonocytic leukemias (CMML). Flow recognition of CMML was initially based on an increase in the fraction of peripheral blood, CD14+,CD16- classical monocytes ≥ 94% of total monocytes. An associated inflammatory disease can preclud...
Source: Best Practice and Research Clinical Haematology - December 7, 2019 Category: Hematology Source Type: research

Myelodysplastic Syndrome/Myeloproliferative Neoplasm Overlap Syndromes – Advances in Treatment
Publication date: Available online 3 December 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Kristen B. McCullough, Mrinal M. PatnaikAbstractOptimal treatment for myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) overlap syndromes remain to be defined and are currently extrapolated from MDS and MPN. The heterogeneity of these diseases and their rare occurrences add to this void. Supportive care therapies such as erythropoiesis stimulating agents, iron chelation and cytoreductive therapy do not have prospective evidence in these disorders and the only approved treatments, hypomethylating ag...
Source: Best Practice and Research Clinical Haematology - December 5, 2019 Category: Hematology Source Type: research

Editorial Board / Aims & Scope
Publication date: December 2019Source: Best Practice & Research Clinical Haematology, Volume 32, Issue 4Author(s): (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - November 26, 2019 Category: Hematology Source Type: research

Index
Publication date: December 2019Source: Best Practice & Research Clinical Haematology, Volume 32, Issue 4Author(s): (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - November 26, 2019 Category: Hematology Source Type: research

Introducing article numbering to Best Practice & Research Clinical Haematology
Publication date: Available online 2 November 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - November 4, 2019 Category: Hematology Source Type: research

What is the role of the microenvironment in MDS?
Publication date: Available online 28 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Laura M. Calvi, Allison J. Li, Michael W. BeckerAbstractTreating myelodysplastic syndromes (MDS) can be complex. Hematopoiesis occurs within a heterogeneous and complex dynamic microenvironment, and a multiplicity of mutations in hematopoietic stem and progenitor cells (HSPCs) lead to MDS. But is there a role for the microenvironment? Here we review experimental and conceptual arguments that support a role for the microenvironment, provide evidence for the disruption of the microenvironment in MDS, and explore ...
Source: Best Practice and Research Clinical Haematology - October 29, 2019 Category: Hematology Source Type: research

Which novel agents hold the greatest promise in AML?
Publication date: Available online 18 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Courtney D. DiNardoAbstractThe therapeutic landscape for acute myeloid leukemia (AML) has changed dramatically with the approval of targeted agents, including venetoclax, midostaurin, gilteritinib, ivosidenib, and enasidenib, among others. However, older patients with AML continue to experience poorer outcomes and are in ongoing need of more effective and less toxic regimens. This review examines the efficacy of novel therapeutics and promising combination approaches to further improve outcomes in the treatment...
Source: Best Practice and Research Clinical Haematology - October 26, 2019 Category: Hematology Source Type: research

Is post-transplant cyclophosphamide a true game-changer in allogeneic transplantation: the struggle to unlearn
Publication date: Available online 20 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Richard J. JonesAbstractClose HLA matching of donors and recipients has been the dogma for successful allogeneic blood or marrow transplantation (BMT), to limit the complications of graft rejection and graft-versus-host disease (GVHD). However, many patients in need, especially those in certain racial and ethnic groups such as African-Americans and Hispanics, are unable to find matches despite increased availability of unrelated donors. Unfortunately, despite many early attempts to develop safe, related haploid...
Source: Best Practice and Research Clinical Haematology - October 21, 2019 Category: Hematology Source Type: research

Acute myeloid leukemia: How to treat the fit patient over age 75?
Publication date: Available online 19 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Selina LugerAbstractSurvival rates for patients with acute myeloid leukemia (AML) older than 75 years are still quite dismal. Recent approvals, therefore, of two agents specifically to treat older patients—glasdegib and venetoclax—have created excitement among the medical community. Clinical data, particularly complete response (CR) rates and CR with incomplete hematologic recovery (CRi), look quite promising and are reviewed here. Yet the question remains whether fit elderly patients should receive combina...
Source: Best Practice and Research Clinical Haematology - October 20, 2019 Category: Hematology Source Type: research

Beyond midostaurin: Which are the most promising FLT3 inhibitors in AML?
Publication date: Available online 18 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Eunice S. WangAbstractMutations of FLT3 occur in around a third of acute myeloid leukemia (AML) patients and are associated with poor outcomes. Multiple targeted tyrosine kinase inhibitors (TKI) have been developed with different selectivity and potency for FLT3 mutant clones. Indications for which FLT3-inhibitor to use depend on the clinical setting and disease status. Patients with relapsed or refractory AML benefit from a different TKI than those with de novo AML or following stem cell transplant. Moreover, ...
Source: Best Practice and Research Clinical Haematology - October 19, 2019 Category: Hematology Source Type: research

Post-remission therapy in acute myeloid leukemia: Are we ready for an individualized approach?
Publication date: Available online 18 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Benjamin A. Derman, Richard A. LarsonAbstractRecent advances in remission induction treatment strategies for acute myeloid leukemia (AML) have improved the rates of complete remission (CR) and overall survival (OS), owing to a concerted effort to tailor therapies toward specific AML subtypes. However, without effective post-remission therapy, most patients will relapse. The extent to which post-remission therapies is individualized in the current paradigm is quite varied. Core binding factor (CBF) AML is typica...
Source: Best Practice and Research Clinical Haematology - October 18, 2019 Category: Hematology Source Type: research

Does mutational burden add to other prognostic factors in MDS?
Publication date: Available online 18 October 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Aziz NazhaAbstractMyelodysplastic syndromes (MDS) are clonal bone marrow disorders characterized by complex genomic abnormalities that define disease phenotype, prognosis, and progression. The overall outcomes of MDS patients are very heterogeneous and can be measured in months in some patients and years in others. Several scoring systems have been developed in MDS, with the International Prognostic Scoring System (IPSS) and its revised version (IPSS-R) the most widely accepted risk stratification tools in clin...
Source: Best Practice and Research Clinical Haematology - October 18, 2019 Category: Hematology Source Type: research