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Large Granular Lymphocytic and Other Rare Lymphoid Leukemias
Publication date: Available online 4 September 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Thomas P. Loughran, Thierry Lamy (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - September 5, 2019 Category: Hematology Source Type: research

T cell large granular lymphocyte leukemia and chronic NK lymphocytosis
Publication date: Available online 22 June 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Gregorio Barilà, Giulia Calabretto, Antonella Teramo, Cristina Vicenzetto, Vanessa Rebecca Gasparini, Gianpietro Semenzato, Renato ZambelloAbstractLarge Granular Lymphocyte Leukemia (LGLL) is a rare chronic lymphoproliferative disorder characterized by the clonal expansion of Large Granular Lymphocytes (LGLs). Among LGLL, the 2016 WHO classification recognizes two different entities, i.e. T-LGLL and the provisional entity Chronic Lymphoproliferative disorder of NK cells (CLPD-NK). In both subtypes neutropenia rep...
Source: Best Practice and Research Clinical Haematology - June 25, 2019 Category: Hematology Source Type: research

Index
Publication date: June 2019Source: Best Practice & Research Clinical Haematology, Volume 32, Issue 2Author(s): (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - June 14, 2019 Category: Hematology Source Type: research

Editorial Board / Aims & Scope
Publication date: June 2019Source: Best Practice & Research Clinical Haematology, Volume 32, Issue 2Author(s): (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - June 14, 2019 Category: Hematology Source Type: research

NK/T-cell lymphomas
Publication date: Available online 12 June 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Eric Tse, Yok-Lam KwongAbstractNK/T-cell lymphomas are extranodal EBV-related malignancies, mostly of NK-cell and occasionally of T-cell lineage. They are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nose, nasopharynx and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other sites. Disseminated NK/T-cell lymphoma involves multiple organs, and may present with a leukemic phase. Initial evaluation req...
Source: Best Practice and Research Clinical Haematology - June 13, 2019 Category: Hematology Source Type: research

Monoclonal B-cell lymphocytosis
Publication date: Available online 7 June 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Elsa Maitre, Xavier TroussardAbstractFlow cytometry diagnostic practices can detect very low levels of clonal B cells in the peripheral blood. In the absence of clinical symptoms, cytopenia or organomegaly, the small clones may correspond to monoclonal B-cell leukemia (MBL) diagnosis. Most MBLs harbor a chronic lymphocytic leukemia (CLL) phenotype (e.g., CD5+, CD23+) and are referred to as CLL-type MBL. The two other types are atypical CLL-type MBL and non-CLL-type MBL. In addition to the phenotypical classificatio...
Source: Best Practice and Research Clinical Haematology - June 8, 2019 Category: Hematology Source Type: research

B and T cell Prolymphocytic leukaemia
Publication date: Available online 6 June 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): M. Cross, C. DeardenAbstractProlymphocytic leukaemias B-PLL and T-PLL are rare disorders, typically with an aggressive clinical course and poor prognosis. Combining morphology, immunophenotyping, cytogenetic and molecular diagnostics reliably separates B-PLL and T-PLL from one another and other disorders. In T-PLL discovery of frequent mutations in the JAK-STAT pathway have increased understanding of disease pathogenesis. Alemtuzumab (anti-CD52) produces excellent response rates but long-term remissions are only ac...
Source: Best Practice and Research Clinical Haematology - June 6, 2019 Category: Hematology Source Type: research

Genomics of LGL Leukemia and select other rare leukemia/lymphomas
We examined the linkage between these mutations and autoimmune symptoms and disorders, in cases of obvious and suspected LGL leukemia. We then summarized and compared mutations in a set of other rare leukemias that also have JAK/STAT signaling pathway activation brought about by genomic changes. These include T-cell acute lymphoblastic leukemia (T-ALL), T-cell prolymphocytic leukemia (T-PLL), cutaneous T-cell lymphoma (CTCL), select peripheral T-cell lymphoma (PTCL), and adult T-cell leukemia/lymphoma (ATLL). Though STAT3 activation is common in these leukemias, the way in which it is achieved, such as the activating cytok...
Source: Best Practice and Research Clinical Haematology - June 6, 2019 Category: Hematology Source Type: research

Leukaemic variants of cutaneous t-cell lymphoma: Erythrodermic mycosis fungoides and Sézary syndrome
Publication date: Available online 6 June 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Xochiquetzal U. Martinez, Cosimo Di Raimondo, Farah R. Abdulla, Jasmine Zain, Steven T. Rosen, Christiane QuerfeldAbstractMycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas. Diagnosis requires correlation of clinical, histologic, and molecular features. A multitude of factors have been linked to the aetiopathogenesis, however, none have been definitively proven. Erythrodermic MF (E-MF) and SS share overlapp...
Source: Best Practice and Research Clinical Haematology - June 6, 2019 Category: Hematology Source Type: research

AML: The Future is Now or was it Yesterday?
Publication date: Available online 25 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Naval Daver, Eytan Stein (Source: Best Practice and Research Clinical Haematology)
Source: Best Practice and Research Clinical Haematology - May 26, 2019 Category: Hematology Source Type: research

Venetoclax-based Therapies for Acute Myeloid Leukemia
Publication date: Available online 24 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Veronica A. Guerra, Courtney DiNardo, Marina KonoplevaAbstractThe prognosis of adult acute myeloid leukemia (AML) remains poor, with the long-term survival rate less than 50%. However, the current paradigms of treatment are changing through a better understanding of the disease genetics and pathophysiology. Since 2017, eight new drugs have been approved by the U.S. Food and Drug Administration for the treatment of AML, including the FLT3 inhibitors midostaurin and gilteritinib, the IDH inhibitors ivosidenib and ena...
Source: Best Practice and Research Clinical Haematology - May 25, 2019 Category: Hematology Source Type: research

Clonal Hematopoiesis and Risk of Acute Myeloid Leukemia
Publication date: Available online 24 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Pinkal Desai, Duane Hassane, Gail J. RobozAbstractAcute Myeloid Leukemia, the most common form of acute leukemia in adults, is an aggressive hematopoietic stem cell malignancy that is associated with significant morbidity and mortality. Though AML generally presents de novo, risk factors include exposure to chemotherapy and/or radiation, as well as both familial and acquired bone marrow failure syndromes. Clonal Hematopoiesis (CH) refers to an expansion of blood or marrow cells resulting from somatic mutations in l...
Source: Best Practice and Research Clinical Haematology - May 25, 2019 Category: Hematology Source Type: research

Daunorubicin-Cytarabine Liposome (CPX-351) in the Management of Newly Diagnosed Secondary AML: A New Twist on an Old Cocktail
Publication date: Available online 13 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Joseph E. Maakaron, Alice S. MimsAbstractInitial therapy for acute myeloid leukemia (AML) remained stagnant for approximately four decades despite advances in improved understanding of pathogenesis and prognostication of the disease. The cornerstone of AML treatment has historically included three days of an anthracycline (daunorubicin or idarubicin) combined with continuous infusion of cytarabine for 7 days, the “7 + 3” regimen. Several attempts have been made to improve on this regimen by dose-intensification...
Source: Best Practice and Research Clinical Haematology - May 14, 2019 Category: Hematology Source Type: research

Incorporating FLT3 inhibitors in the frontline treatment of FLT3 mutant acute myeloid leukemia
Publication date: Available online 12 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Eunice S. WangAbstractFLT3 mutations occur in up to a third of newly diagnosed patients with acute myeloid leukemia (AML) and confer poor prognosis. Clinical development of FLT3 tyrosine kinase inhibitors for AML initially involved broad-spectrum inhibitors (midostaurin, sorafenib) targeting multiple kinases. Addition of midostaurin to upfront intensive chemotherapy for younger patients with FLT3 mutant AML significantly improved overall survival and validated FLT3 as a therapeutic target. Other regimens such as so...
Source: Best Practice and Research Clinical Haematology - May 14, 2019 Category: Hematology Source Type: research

Can we incorporate MRD assessment into clinical practice in AML?
Publication date: Available online 10 May 2019Source: Best Practice & Research Clinical HaematologyAuthor(s): Gert Ossenkoppele, Gerrit Jan Schuurhuis, Arjan van de Loosdrecht, Jacqueline CloosAbstractMeasurable residual disease (MRD) can be assessed either by flow cytometry or molecular techniques. It has been proven to be highly prognostic in quite a number of prospective clinical studies. The recently published ELN MRD recommendations aim harmonize the approaches to MRD assessment in order to improve its overall quality. The predictive value leading to the usage as a surrogate endpoint for survival which would be instru...
Source: Best Practice and Research Clinical Haematology - May 11, 2019 Category: Hematology Source Type: research