Challenges in Chronic Myeloid Leukemia Management in South America
AbstractPurpose of ReviewChronic myeloid leukemia (CML) management in developing countries has improved in the last years, but the availability of therapeutic resources, monitoring, reimbursement, and financial issues may be a challenge and interfere with the best practices and results of CML treatment. This review points out the main challenges in CML management in South America.Recent Findings.In this review, we describe the access to tyrosine kinase inhibitors and monitoring in different countries of South America. We also address the ongoing discontinuation trials, the progress, and limitations of hematopoietic stem ce...
Source: Current Hematologic Malignancy Reports - October 1, 2021 Category: Hematology Source Type: research

Application of Next-Generation Sequencing-Based Mutational Profiling in Acute Lymphoblastic Leukemia
This article identifies avenues within the landscape of adult acute lymphoblastic leukemia (ALL) where mutational data hold the opportunity to enhance understanding of disease biology and patient care.Recent FindingsNGS-based assessment of measurable residual disease (MRD) after ALL treatment allows for a sensitive and specific molecular survey that is at least comparable, if not superior, to existing techniques. Mutational assessment by NGS has unraveled complex signaling networks that drive pathogenesis of T-cell ALL. Sequencing of patients with familial clustering of ALL has also identified novel germline mutations whos...
Source: Current Hematologic Malignancy Reports - October 1, 2021 Category: Hematology Source Type: research

Treatment-Free Remission: the New Goal in CML Therapy
AbstractPurpose of ReviewTreatment-free remission (TFR) is considered one of the main goals of therapy in patients with CML. Our goal in this paper is to review the current data on TFR, and discuss future directions.Recent FindingsMultiple studies have demonstrated that attempting a treatment-free remission is safe and effective in a select group of patients. More recent data suggested that undetectable BCR-ABL1 by digital PCR prior to discontinuation is highly predictive of successful TFR. However, some patients have a successful TFR with no evidence of clinical disease despite persistent detectable BCR-ABL1. Some recent ...
Source: Current Hematologic Malignancy Reports - October 1, 2021 Category: Hematology Source Type: research

The Evolving Landscape of Frontline Therapy in Chronic Phase Chronic Myeloid Leukemia (CML)
AbstractPurpose of ReviewChronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by uncontrolled proliferation of mature and maturing granulocytes. The disease is characterized by the presence of translocation t(9;22) leading to the abnormal BCR-ABL fusion. Historically, treatment options included hydroxyurea, busulfan, and interferon- α (IFN-α), with allogeneic stem cell transplant being the only potential curative therapy. More recently, the development of tyrosine kinase inhibitors (TKIs) has revolutionized the treatment of CML and turned a once fatal disease into a chronic and manageable disorde...
Source: Current Hematologic Malignancy Reports - October 1, 2021 Category: Hematology Source Type: research

Philadelphia-Negative Myeloproliferative Neoplasms Around the COVID-19 Pandemic
AbstractPurpose of ReviewCoronavirus disease 2019 (COVID-19) is associated with a high rate of respiratory failure, thromboembolism, bleeding, and death. Patients with myeloproliferative neoplasms (MPNs) are prone to both thrombosis and bleeding, calling for special care during COVID-19. We reviewed the clinical features of MPN patients with COVID-19, suggesting guidance for treatment.Recent FindingsOne study by the European LeukemiaNet collected 175 MPN patients with COVID-19 during the first wave of the pandemic, from February to May 2020. Patients with primary myelofibrosis (PMF) were at higher risk of mortality (48%) i...
Source: Current Hematologic Malignancy Reports - September 29, 2021 Category: Hematology Source Type: research

Elevating Twitter-Based Journal Club Discussions by Leveraging a Voice-Based Platform: #HemepathJC Meets Clubhouse
AbstractPurpose of ReviewSocial media-based scientific journal clubs provide an opportunity to promote published literature to a broader audience and allow robust multi-disciplinary and inter-professional discussion. Hematopathology Journal Club (#HemepathJC) on Twitter has successfully conducted monthly sessions since November 2019, covering topics related to lymphoma and leukemia.Recent FindingsTo enhance connectivity, multitasking, and productivity, we present our experience of leveraging the voice-based platform Clubhouse concurrent with Twitter.SummaryThe Twitter and Clubhouse partnership for #hemepathJC holds the pot...
Source: Current Hematologic Malignancy Reports - September 29, 2021 Category: Hematology Source Type: research

Philadelphia-Negative Myeloproliferative Neoplasms Around the COVID-19 Pandemic
AbstractPurpose of ReviewCoronavirus disease 2019 (COVID-19) is associated with a high rate of respiratory failure, thromboembolism, bleeding, and death. Patients with myeloproliferative neoplasms (MPNs) are prone to both thrombosis and bleeding, calling for special care during COVID-19. We reviewed the clinical features of MPN patients with COVID-19, suggesting guidance for treatment.Recent FindingsOne study by the European LeukemiaNet collected 175 MPN patients with COVID-19 during the first wave of the pandemic, from February to May 2020. Patients with primary myelofibrosis (PMF) were at higher risk of mortality (48%) i...
Source: Current Hematologic Malignancy Reports - September 29, 2021 Category: Hematology Source Type: research

Elevating Twitter-Based Journal Club Discussions by Leveraging a Voice-Based Platform: #HemepathJC Meets Clubhouse
AbstractPurpose of ReviewSocial media-based scientific journal clubs provide an opportunity to promote published literature to a broader audience and allow robust multi-disciplinary and inter-professional discussion. Hematopathology Journal Club (#HemepathJC) on Twitter has successfully conducted monthly sessions since November 2019, covering topics related to lymphoma and leukemia.Recent FindingsTo enhance connectivity, multitasking, and productivity, we present our experience of leveraging the voice-based platform Clubhouse concurrent with Twitter.SummaryThe Twitter and Clubhouse partnership for #hemepathJC holds the pot...
Source: Current Hematologic Malignancy Reports - September 29, 2021 Category: Hematology Source Type: research

Avapritinib in the Treatment of Systemic Mastocytosis: an Update
AbstractPurpose of ReviewPatients with systemic mastocytosis, a dangerous and rare myeloid neoplasm, have long had few therapies available to them and, historically, rarely achieved from significant disease control. However, research and translational developments over the last decade have led to promising new options for disease management. In this review, we briefly outline the history of treatment for systemic mastocytosis and subsequently focus on the clinical development and potential applications of avapritinib (previously known as BLU-285), a potent and selective oral inhibitor of the tyrosine kinase most commonly m...
Source: Current Hematologic Malignancy Reports - September 27, 2021 Category: Hematology Source Type: research

Management of Myelofibrosis-Associated Anemia: Focus on Standard Agents and Novel Therapeutics in Phase 3 Clinical Trials
AbstractPurpose of ReviewThe management of myelofibrosis is risk-adapted when considering transplant eligibility and symptom-directed, prioritizing the most burdensome symptoms for the patient. Unfortunately, myelofibrosis-anemia is common, multifactorial in its origin, and impactful regarding prognosis. While clinical trials are advised, not all patients have convenient access, and therefore, hematologists should be aware of the data supporting the use of conventional agents such as erythropoietin-stimulating agents, steroid treatments (danazol and prednisone), and immunomodulatory drugs (thalidomide and lenalidomide). Th...
Source: Current Hematologic Malignancy Reports - September 9, 2021 Category: Hematology Source Type: research

Management and Outcomes of Blast Transformed Chronic Myelomonocytic Leukemia
AbstractPurpose of ReviewDespite recent advances in the treatment of de novo acute myeloid leukemia (AML), AML arising from antecedent chronic myelomonocytic leukemia (CMML) continues to have dismal outcomes. While the unique biological drivers of CMML and subsequent leukemic transformation (LT) have been revealed with advances in molecular characterization, this has not yet translated to the bedside. Here, we review these biologic drivers, outcomes with current therapies, and rationale avenues of future investigation specifically in blast phase CMML (CMML-BP).Recent FindingsCMML-BP outcomes are studied as an aggregate wit...
Source: Current Hematologic Malignancy Reports - September 9, 2021 Category: Hematology Source Type: research

Management of Myelofibrosis-Associated Anemia: Focus on Standard Agents and Novel Therapeutics in Phase 3 Clinical Trials
AbstractPurpose of ReviewThe management of myelofibrosis is risk-adapted when considering transplant eligibility and symptom-directed, prioritizing the most burdensome symptoms for the patient. Unfortunately, myelofibrosis-anemia is common, multifactorial in its origin, and impactful regarding prognosis. While clinical trials are advised, not all patients have convenient access, and therefore, hematologists should be aware of the data supporting the use of conventional agents such as erythropoietin-stimulating agents, steroid treatments (danazol and prednisone), and immunomodulatory drugs (thalidomide and lenalidomide). Th...
Source: Current Hematologic Malignancy Reports - September 9, 2021 Category: Hematology Source Type: research

Management and Outcomes of Blast Transformed Chronic Myelomonocytic Leukemia
AbstractPurpose of ReviewDespite recent advances in the treatment of de novo acute myeloid leukemia (AML), AML arising from antecedent chronic myelomonocytic leukemia (CMML) continues to have dismal outcomes. While the unique biological drivers of CMML and subsequent leukemic transformation (LT) have been revealed with advances in molecular characterization, this has not yet translated to the bedside. Here, we review these biologic drivers, outcomes with current therapies, and rationale avenues of future investigation specifically in blast phase CMML (CMML-BP).Recent FindingsCMML-BP outcomes are studied as an aggregate wit...
Source: Current Hematologic Malignancy Reports - September 9, 2021 Category: Hematology Source Type: research

Management Issues and Controversies in Low-Risk Patients with Essential Thrombocythemia and Polycythemia Vera
AbstractPurpose of ReviewEssential thrombocythemia (ET) and polycythemia vera (PV) are the most common myeloproliferative neoplasms (MPNs). Treatment of ET and PV is based on the risk for subsequent thrombosis. High-risk patients, defined as older than 60,JAK2 V617F–positive patients, or patients with a history of prior thrombosis, merit cytoreduction to control blood counts, whereas a watchful waiting paradigm is utilized in low-risk patients. However, low-risk patients have a host of other specific management issues that arise during their disease course. This review will discuss the most common management issues speci...
Source: Current Hematologic Malignancy Reports - September 3, 2021 Category: Hematology Source Type: research