Myeloproliferative Neoplasms in Children, Adolescents, and Young Adults
AbstractPurpose of ReviewMyeloproliferative neoplasms are traditionally seen in older adults, making them poorly understood in younger patients. Clinical presentation, genetic landscape, outcomes, and best management practices are inadequately described in this group. Over the past decade, more research has focused on younger patients, and this paper seeks to review and describe the current status of the field.Recent FindingsA recent review analyzed the available pediatric MPN literature and highlighted the paucity of published data. Pediatric patients showed lower rates of the common mutations found in adults, thrombotic ...
Source: Current Hematologic Malignancy Reports - March 13, 2020 Category: Hematology Source Type: research

Indications for Allogeneic Hematopoietic Cell Transplantation in Myelodysplastic Syndrome
AbstractPurpose of ReviewMyelodysplastic syndromes (MDS) are heterogeneous diseases that principally affect older adults. Allogeneic hematopoietic cell transplantation (HCT) is the only potentially curative therapy; however, non-relapse mortality (NRM) accounts for as many as 40% of deaths after HCT and underscores the need for careful patient selection. We review the common indications and causes of failure after HCT in MDS.Recent FindingsAppropriate patient selection is necessary to optimize HCT outcomes and maximize the life-expectancies of MDS patients. The international prognostic scoring systems (IPSS) and revised IP...
Source: Current Hematologic Malignancy Reports - March 2, 2020 Category: Hematology Source Type: research

Is There a Role for Chemotherapy in the Era of Targeted Therapies?
AbstractPurpose of ReviewThe treatment landscape of chronic lymphocytic leukemia has been rapidly evolving over the past few years. The prior standard of care, chemoimmunotherapy, is being replaced by targeted agents, and the utility of chemotherapy has come under question. In this review, we examine recent data comparing chemoimmunotherapy to targeted agents, how these data impact clinical management, and whether there are potential future roles for cytotoxic chemotherapy.Recent FindingsClinical trials have shown improved clinical outcomes with targeted agents compared to traditional chemoimmunotherapy. Based on these dat...
Source: Current Hematologic Malignancy Reports - February 26, 2020 Category: Hematology Source Type: research

Mechanisms of Cardiovascular Toxicity of BCR-ABL1 Tyrosine Kinase Inhibitors in Chronic Myelogenous Leukemia
AbstractPurpose of ReviewOral tyrosine kinase inhibitors have revolutionized the treatment of chronic myelogenous leukemia, with many patients achieving major clinical and molecular responses without complications. While typically well-tolerated, clinical experience with tyrosine kinase inhibitors (particularly those of the second and third generations) has highlighted unanticipated associations with serious non-cancer adverse effects on various organs, particularly the cardiovascular system.Recent FindingsHerein, we review the current literature surrounding the major cardiovascular toxicities of BCR-ABL1 tyrosine kinase i...
Source: Current Hematologic Malignancy Reports - February 19, 2020 Category: Hematology Source Type: research

Advances in the Diagnosis and Treatment of Large Granular Lymphocytic Leukemia
AbstractPurpose of ReviewThe past decade in LGL leukemia research has seen increased pairing of clinical data with molecular markers, shedding new insights on LGL leukemia pathogenesis and heterogeneity. This review summarizes the current standard of care of LGL leukemia, updates from clinical trials, and our congruent improved understanding of LGL pathogenesis.Recent FindingsVarious clinical reports have identified associations between stem, bone marrow, and solid organ transplants and incidence of LGL leukemia. There is also a potential for underdiagnosis of LGL leukemia within the rheumatoid arthritis patient population...
Source: Current Hematologic Malignancy Reports - February 14, 2020 Category: Hematology Source Type: research

Patient-Reported Outcomes in Myelodysplastic Syndromes: the Move from Life Span to Health Span
AbstractPurpose of ReviewThe lack of fully effective therapies to alter the natural course of myelodysplastic syndromes (MDS) leads to chronic morbidity, mortality and affects quality of life (QoL). Since existing therapies outside of hematopoietic cell transplantation (HCT) are not curative, there is a growing interest in incorporating patient-reported outcomes (PROs) as meaningful endpoints for these patients in research and clinical practice.Recent FindingsCurrently, there are limited numbers of studies reporting the impact of MDS therapeutics on PROs to guide clinical decision-making and increase patient satisfaction. ...
Source: Current Hematologic Malignancy Reports - February 10, 2020 Category: Hematology Source Type: research

Novel Therapies in Polycythemia Vera
AbstractPurpose of ReviewPolycythemia vera is a myeloproliferative neoplasm characterized by increased erythrocyte count, thrombotic potential, and transformation to myelofibrosis. Older patients and those who have a history of thrombosis require cytoreductive therapy, most commonly with hydroxyurea. Other currently available therapies include pegylated interferon alfa-2a and the JAK1/2 inhibitor ruxolitinib. However, there are limitations to these agents, including potential detrimental adverse effects. In this review, we will describe current therapeutic options for the treatment of PV and then detail new agents with ava...
Source: Current Hematologic Malignancy Reports - February 6, 2020 Category: Hematology Source Type: research

Advances and Perspectives in the Treatment of T-PLL
AbstractPurpose of ReviewT cell prolymphocytic leukemia (T-PLL) is a rare mature T cell tumor. Available treatment options in this aggressive disease are largely inefficient and patient outcomes are highly dissatisfactory. Current therapeutic strategies mainly employ the CD52-antibody alemtuzumab as the most active single agent. However, sustained remissions after sole alemtuzumab-based induction are exceptions. Responses after available second-line strategies are even less durable. More profound disease control or rare curative outcomes can currently only be expected after a consolidating allogeneic hematopoietic stem cel...
Source: Current Hematologic Malignancy Reports - February 6, 2020 Category: Hematology Source Type: research

Current Approaches to Transplantation for FLT3-ITD AML
AbstractPurpose of ReviewThis review discusses the current standard of care for incorporation of FLT3 TKIs and HCT into the treatment of FLT3-ITD AML. Additionally, this review provides an approach to the patient with relapsed/refractory disease.Recent FindingsOver the last decade, the routine use of HCT as consolidative therapy and the development of FLT3 TKIs have significantly improved remission rates and overall survival. The value and challenges of MRD assessment in FLT3 disease are discussed and current mechanisms of relapse are explored, as are the ongoing questions in the field that current clinical trials are seek...
Source: Current Hematologic Malignancy Reports - February 6, 2020 Category: Hematology Source Type: research

Brentuximab Vedotin in the Treatment of Peripheral T Cell Lymphoma and Cutaneous T Cell Lymphoma
AbstractPurpose of ReviewThe recent development of brentuximab vedotin (BV), an antibody-drug conjugate targeting CD30-positive cells, has led to therapeutic advances in the treatment of T cell lymphomas. In this review, we discuss key studies of BV in peripheral T cell lymphoma (PTCL) and cutaneous T cell lymphoma (CTCL) and highlight important questions for further investigation.Recent FindingsMonotherapy with BV has proven to be effective and well tolerated in patients with relapsed/refractory (R/R) CD30-positive CTCL. BV has shown significant activity in R/R PTCL as well, with particularly durable responses in patients...
Source: Current Hematologic Malignancy Reports - February 2, 2020 Category: Hematology Source Type: research

The Cardiovascular Complications of Chimeric Antigen Receptor T Cell Therapy
AbstractPurpose of ReviewChimeric antigen receptor T cell therapy is gaining clinical use in the management of B cell lymphomas. As the use of this unique treatment option increases, its associated toxicities will require recognition and treatment. In this review, we aim to discuss the cardiovascular toxicities of chimeric antigen receptor T cell therapy and our approach to their clinical management.Recent FindingsCardiotoxicity may be due to direct or indirect effects of infused chimeric antigen receptor T cells. The cytokine release syndrome has been described extensively in the literature. Studies have also reported car...
Source: Current Hematologic Malignancy Reports - February 2, 2020 Category: Hematology Source Type: research

Novel Treatment Strategies in the Management of Waldenstr öm Macroglobulinemia
AbstractPurpose of ReviewRecent advances the genomic profiling of patients with Waldenstr öm macroglobulinemia (WM) have led to the identification of novel therapeutic targets in these patients. In this review, we cover the current standard of care and the recently evaluated novel approaches with high potential to be incorporated in the therapeutic armamentarium against WM.Recent FindingsTheMYD88L265P mutation is the most common genomic abnormality in WM, and is encountered in 80 –95% of patients, making it an important target for drug development. The success of the first-generation Bruton tyrosine kinase (BTK) inhibit...
Source: Current Hematologic Malignancy Reports - January 30, 2020 Category: Hematology Source Type: research

Moving Past Ganciclovir and Foscarnet: Advances in CMV Therapy
AbstractPurpose of ReviewCMV DNA polymerase inhibitors such as ganciclovir and foscarnet have dramatically reduced the burden of CMV infection in the HCT recipient. However, their use is often limited by toxicities and resistance. Agents with novel mechanisms and favorable toxicity profiles are critically needed. We review recent developments in CMV antivirals and immune-based approaches to mitigating CMV infection.Recent FindingsLetermovir, an inhibitor of the CMV terminase complex, was approved in 2017 for primary CMV prophylaxis in adult seropositive allogeneic HCT recipients. Maribavir, an inhibitor of the CMV UL97 kin...
Source: Current Hematologic Malignancy Reports - January 23, 2020 Category: Hematology Source Type: research

What Does Chronic Myeloid Leukaemia Tell Us About Other Leukaemias?
AbstractPurpose of ReviewDetermine if therapy of chronic myeloid leukaemia (CML) is a model for treating other cancers.Recent FindingsCML has a relatively homogeneous phenotype and genotype and is caused by one mutation,BCRABL1, in every instance. In contrast, most other leukaemias, haematologic cancers and solid cancer have more heterogeneous phenotypes and extraordinarily greater genotypic diversity and mutational complexity.SummaryLesions learned from treating CML have little applicability to other leukaemias, haematologic cancers or solid cancer. (Source: Current Hematologic Malignancy Reports)
Source: Current Hematologic Malignancy Reports - December 18, 2019 Category: Hematology Source Type: research

Updates in Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)
AbstractPurpose of ReviewBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that has heterogeneous presentation and can involve the skin, lymph nodes, and bone marrow. Recent advancements in our patho-biologic understanding of the disease have led to the development of new targeted therapies for BPDCN. In this review, we aimed to describe some of the novel treatments that are being put forward for the management of BPDCN.Recent FindingsTagraxofusp is the first CD123-targeted therapy approved as the first ever targeted treatment of BPDCN in patients aged 2  years an...
Source: Current Hematologic Malignancy Reports - December 17, 2019 Category: Hematology Source Type: research