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Targeting BCMA in Multiple Myeloma
AbstractPurpose of ReviewDespite considerable advances in the treatment of multiple myeloma (MM) in the last decade, a significant number of patients still progress on current available therapies. Here, we review treatment modalities used to target BCMA in the treatment of MM, specifically antibody-drug conjugates (ADC), bispecific antibody constructs, and chimeric antibody receptor (CAR) modified T-cell therapies. We will provide an overview of therapies from these classes that have presented or published clinical data, as well as data on mechanisms of resistance to these novel agents.Recent FindingsClinical trials explor...
Source: Current Hematologic Malignancy Reports - August 25, 2021 Category: Hematology Source Type: research

Acute Lymphoblastic Leukemia and Acute Lymphoblastic Lymphoma: Same Disease Spectrum but Two Distinct Diagnoses
AbstractPurpose of ReviewRare malignancies developing from lymphocyte precursor cells, lymphoblastic leukemia (LBL), and acute lymphoblastic lymphoma (ALL) have historically been viewed as different manifestations of the same disease process. This review examines data on their epidemiology, genetics, clinical presentation, and response to treatment while highlighting areas of similarity and divergence between these two clinical entities.Recent FindingsPediatric-type ALL chemotherapy regimens, compared to both lymphoma-type chemotherapy and adult-type ALL regimens, have led to improved outcomes for children, adolescents, an...
Source: Current Hematologic Malignancy Reports - August 21, 2021 Category: Hematology Source Type: research

Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management
AbstractPurpose of ReviewAmyloidosis is a protein deposition disease whereby a variety of precursor proteins form insoluble fibrils that deposit in tissues, causing organ dysfunction and, many times, death. Accurate characterization of the disease based on the nature of the precursor protein, organ involvement, and extent of disease is paramount to guide management. Cardiac amyloidosis is critical to understand because of its impact on prognosis and new treatment options available.Recent FindingsNew imaging methods have proven to be considerably valuable in the identification of cardiac amyloid infiltration. For treating c...
Source: Current Hematologic Malignancy Reports - August 1, 2021 Category: Hematology Source Type: research

Balancing Quality, Cost, and Access During Delivery of Newer Cellular and Immunotherapy Treatments
We describe various novel approaches to manage challenges in improving access and improving widescale implementation of cellular therapies. (Source: Current Hematologic Malignancy Reports)
Source: Current Hematologic Malignancy Reports - August 1, 2021 Category: Hematology Source Type: research

Frontline Treatment for Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL): Targeted Therapy vs. Chemoimmunotherapy
AbstractPurpose of ReviewThe treatment options for chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) have expanded significantly in the last few years, including the use of new classes of oral small molecular inhibitors targeting the B cell receptor signaling pathway or the apoptosis machinery. Targeted therapy with or without immunotherapy has quickly emerged as a new standard for frontline treatment of CLL/SLL, though the previous standard chemoimmunotherapy (CIT) remains a treatment option. In this review, we present data from key clinical trials to evaluate the benefits and risks associated with differe...
Source: Current Hematologic Malignancy Reports - August 1, 2021 Category: Hematology Source Type: research

The Emerging Role of Hematopathologists and Molecular Pathologists in Detection, Monitoring, and Management of Myeloid Neoplasms with Germline Predisposition
AbstractPurpose of ReviewAwareness, widespread availability, and routine use of sequencing techniques in work-up of myelodysplastic syndromes and acute myeloid leukemia have facilitated increased recognition of these entities arising in a background of germline predisposition disorders (GPD).Recent FindingsThe latest revisions to the WHO classification of myeloid neoplasms incorporate “myeloid neoplasms with germline predisposition” as a separate entity due to the therapeutic implications of this diagnosis. It has become apparent that some of these entities have unique recognizable morphologic findings that can be chal...
Source: Current Hematologic Malignancy Reports - August 1, 2021 Category: Hematology Source Type: research

Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management
AbstractPurpose of ReviewAmyloidosis is a protein deposition disease whereby a variety of precursor proteins form insoluble fibrils that deposit in tissues, causing organ dysfunction and, many times, death. Accurate characterization of the disease based on the nature of the precursor protein, organ involvement, and extent of disease is paramount to guide management. Cardiac amyloidosis is critical to understand because of its impact on prognosis and new treatment options available.Recent FindingsNew imaging methods have proven to be considerably valuable in the identification of cardiac amyloid infiltration. For treating c...
Source: Current Hematologic Malignancy Reports - June 9, 2021 Category: Hematology Source Type: research

Balancing Quality, Cost, and Access During Delivery of Newer Cellular and Immunotherapy Treatments
We describe various novel approaches to manage challenges in improving access and improving widescale implementation of cellular therapies. (Source: Current Hematologic Malignancy Reports)
Source: Current Hematologic Malignancy Reports - June 5, 2021 Category: Hematology Source Type: research

Pegaspargase in Practice: Minimizing Toxicity, Maximizing Benefit
AbstractPurpose of ReviewThe incorporation of pegaspargase in chemotherapy regimens has significantly improved the prognosis of ALL in adults. However, pegaspargase use poses many challenges due to its unique toxicity profile. Here, we review pegaspargase ’s most clinically significant toxicities, and provide guidance for their prevention and management in order to avoid unnecessary drug discontinuation and achieve maximum clinical benefit.Recent FindingsClinically significant toxicities of pegaspargase include thrombosis, hypersensitivity and inactivation, hepatotoxicity, pancreatitis, and hypertriglyceridemia. The majo...
Source: Current Hematologic Malignancy Reports - June 1, 2021 Category: Hematology Source Type: research

Novel Pathophysiological Mechanisms of Thrombosis in Myeloproliferative Neoplasms
AbstractPurpose of ReviewThrombosis remains a leading cause of morbidity and mortality in BCR/ABL negative myeloproliferative neoplasms (MPN). Circulating blood cells are both increased in quantity and qualitatively abnormal in MPN, resulting in an increased thrombotic risk. Herein, we review recently elucidated mechanisms of MPN thrombosis and discuss implications of drugs currently under investigation for MPN.Recent FindingsRecent studies highlight that inJAK2V617F granulocytes and platelets, thrombo-inflammatory genes are upregulated. Furthermore, inJAK2V617F granulocytes, protein expression of integrin CD11b, tissue fa...
Source: Current Hematologic Malignancy Reports - June 1, 2021 Category: Hematology Source Type: research

Pediatric Neoplasms Presenting with Monocytosis
AbstractPurpose of ReviewJuvenile myelomonocytic leukemia (JMML) is a rare but severe pediatric neoplasm with hematopoietic stem cell transplant as its only established curative option. The development of targeted therapeutics for JMML is being guided by an understanding of the pathobiology of this condition. Here, we review JMML with an emphasis on genetics in order to (i) demonstrate the relationship between JMML genotype and clinical phenotype and (ii) explore potential genetic targets of novel JMML therapies.Recent FindingsDNA hypermethylation studies have demonstrated consistently that methylation is related to diseas...
Source: Current Hematologic Malignancy Reports - June 1, 2021 Category: Hematology Source Type: research

Clinical and Molecular Approach to Adult-Onset, Neoplastic Monocytosis
AbstractPurpose of ReviewIn this review, we provide a comprehensive and contemporary understanding of malignant monocytosis and provide a framework by which the appropriate diagnosis with malignant monocytosis can be rendered.Recent FindingsIncreasing data support the use of molecular data to refine the diagnostic approach to persistent monocytosis. The absence of aTET2,SRSF2, orASXL1 mutation has ≥ 90% negative predictive value for a diagnosis of CMML. These data may also reliably differentiate chronic myelomonocytic leukemia, the malignancy that is most associated with mature monocytosis, from several other diseases th...
Source: Current Hematologic Malignancy Reports - June 1, 2021 Category: Hematology Source Type: research