Hematology, ASH Education Program | American Society of Hematology

Time 2020-07-07 09:04:31

Web Name: Hematology, ASH Education Program | American Society of Hematology

WebSite: http://asheducationbook.hematologylibrary.org

ID:21344

Keywords:

Education,ASH,Hematology,

Description:

Hematology 2019 provides review articles from the Education Program at the 2019 ASH Annual Meeting. In this publication, each chapter relates to a different session presented at the meeting, spanning the spectrum from basic discovery and disease pathogenesis to the clinical application at the bedside.This activity is supported by educational grants from Amgen, AstraZeneca, Celgene Corporation, Incyte Corporation, Jazz Pharmaceuticals, Inc., Novartis Pharmaceuticals, and Servier Pharmaceuticals, LLC.Navigating the nexus of MRD and novel agents in ALL The landscape of acute lymphoblastic leukemia (ALL) has evolved significantly over the last few years. Identification of specific recurrent genetic alterations and of minimal residual disease (MRD) guides prognostic classification and management. Novel agents (eg, blinatumomab) have demonstrated encouraging results in relapsed/refractory (R/R) and MRD+ patients and are currently incorporated into upfront treatment in specific settings. Other new strategies include the incorporation of tyrosine kinase inhibitor-based therapy for patients with Philadelphia chromosome like ALL and the use of DOT inhibitors and bcl-2/bcl-xl inhibitors in R/R disease. These innovations promise to improve management and outcome in this disease.How I treat a refractory myeloma patient who is not eligible for a clinical trial Myeloma patients not eligible for clinical trials have many treatment options. Choosing the next best therapy starts with careful assessment of the biology and dynamics of the disease at relapse, as well as the condition and situation of the patient. Fit patients should be considered for triplet regimens, whereas intermediate and frail patients warrant dose-reduced triplets or doublets. An indolent serologic relapse may be treated with dose intensification, especially in a maintenance situation, whereas a rapid relapse requires a more aggressive approach with drug class change or a second-generation immunomodulatory drug (IMID) or proteasome inhibitor (PI). Monoclonal antibodies, in combination with PIs and IMIDs, have proven highly efficacious in early and late relapse. Key elements of supportive care include infection prevention, bone health, thromboprophylaxis, and management of active symptoms, such as pain and distress.Idiopathic aplastic anemia vs hypocellular myelodysplastic syndromeProper diagnostic distinction of bone marrow failure syndromes can often be challenging. In particular, for older patients with idiopathic aplastic anemia (AA), differential diagnosis includes myelodysplastic syndrome (MDS), which can atypically present in a hypocellular form. In addition to blasts and overt dysplasia, the presence of chromosomal abnormalities and a spectrum of somatic mutations may be revealing. Both clonal cytogenetic aberrations and somatic mutations most typically correspond to a clonal myelodysplasia, but clonal somatic mutations have also recently been found in AA. True driver myeloid mutations are uncommon in AA. Marrow hypocellularity in AA and occasionally in MDS patients points toward a similar immune mechanism responsible for deficient blood cell production and indicates that cytopenias in early hypocellular MDS might be treated with immunosuppressive modalities. Primary hypocellular MDS has to be distinguished from post-AA secondary MDS, most commonly associated with del7/7q. Post-AA MDS evolves at the rate of about 10% in 10 years, but recent observations suggest that widespread use of eltrombopag may influence the risk of progression to MDS. This complication likely represents a clonal escape, with founder hits occurring early on in the course of AA. A similar mechanism operates in the evolution of paroxysmal nocturnal hemoglobinuria (PNH) in AA patients, but PNH clones are rarely encountered in primary MDS.

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