New Approaches to Treating Challenging Subtypes of ALL in AYA Patients
AbstractPurpose of ReviewThe treatment of acute lymphoblastic leukemia (ALL) in adolescent and young adult (AYA) patients has markedly improved with the adoption of pediatric-inspired protocols. However, there remain several subtypes of ALL that represent significant therapeutic challenges. Here, we review the current evidence guiding treatment of Philadelphia chromosome-positive (Ph+), Philadelphia chromosome-like (Ph-L), and early T-precursor (ETP) ALL in the AYA population.Recent FindingsClinical trials in Ph + ALL have demonstrated the superior efficacy of second- and third-generation tyrosine kinase inhibitors (TKIs) ...
Source: Current Hematologic Malignancy Reports - September 11, 2020 Category: Hematology Source Type: research

Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splenic Radiation in 2020?
AbstractPurpose of ReviewMyelofibrosis is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly. Currently approved medical therapies do not improve splenomegaly in all patients and effects are not sustained. Thus, spleen-directed therapies (i.e., splenectomy and splenic irradiation) have been used in some cases to palliate the signs and symptoms of the disease. Here, we critically review the literature regarding palliative splenectomy and splenic irradiation in myelofibrosis, and discuss their position in the current treatment landscape.Recent FindingsRetrospective studies have demonstrated that spl...
Source: Current Hematologic Malignancy Reports - August 21, 2020 Category: Hematology Source Type: research

The Next Generation of JAK Inhibitors: an Update on Fedratinib, Momelotonib, and Pacritinib
AbstractPurpose of ReviewRuxolitinib is the first FDA-approved JAK inhibitor for the treatment of myeloproliferative neoplasms and is an effective means of controlling symptom burden and improving splenomegaly. However, a majority of patients will develop disease progression with long-term use. Fedratinib, momelotinib, and pacritinib are three newer-generation JAK inhibitors being prospectively evaluated and we will discuss their roles in the treatment of myeloproliferative neoplasms.Recent FindingsFedratinib has a role in both JAK-inhibitor naive intermediate-/high-risk myelofibrosis patients and in patients that have pre...
Source: Current Hematologic Malignancy Reports - August 10, 2020 Category: Hematology Source Type: research

Preparing Trainees to Deliver High-Value and Cost-Conscious Care in Hematology
AbstractPurpose of ReviewDespite national-level directives to reduce healthcare waste and promote high-value care (HVC), clinical educators struggle to equip trainees with the knowledge and skills needed to practice value-based care. In this review, we analyze ongoing efforts in graduate medical education (GME) to enhance trainee competence in delivery of high-value and cost-conscious care.Recent FindingsSurveys of residents and program directors have shown that while many training programs want to offer formal training in high-value care delivery, few succeed. Although several studies suggest that trainees model stewardsh...
Source: Current Hematologic Malignancy Reports - July 5, 2020 Category: Hematology Source Type: research

Advances in Supportive Care for Acute Lymphoblastic Leukemia
This article will review the novel therapies in acute leukemias and highlight the clinically relevant supportive care advances.Recent FindingsThe American Society for Transplantation and Cellular Therapy (ASTCT) has put forth the most recent recommendations in managing the cytokine release syndrome and neurotoxicity after chimeric antigen receptor T cells (CAR-T) and blinatumomab. The hepatic injury incurred by inotuzumab, and the vascular toxicity of tyrosine kinase inhibitors, other relatively novel agents, require subspecialist intervention and multidisciplinary care. Asparaginase, a long-established and key element of ...
Source: Current Hematologic Malignancy Reports - June 29, 2020 Category: Hematology Source Type: research

Frontline Therapy of Chronic Lymphocytic Leukemia: Changing Treatment Paradigm
AbstractPurpose of ReviewThe treatment landscape of treatment-naive chronic lymphocytic leukemia (TN-CLL) is rapidly evolving. As more and more new drugs and combinations are becoming part of therapeutic armamentarium, it becomes highly pertinent to understand the evidence for each of the treatment options to select the right drug for the right patient. We summarize the recent data of the available frontline treatment options.Recent FindingsThe novel agents can overcome adverse biological attributes and provide long-term disease control. MRD may become a reliable surrogate for survival in the evaluation of future therapies...
Source: Current Hematologic Malignancy Reports - June 14, 2020 Category: Hematology Source Type: research

Immunodeficiency-Related Lymphoid Proliferations: New Insights With Relevance to Practice
AbstractPurpose of ReviewOur understanding of risk factors and mechanisms underlying immunosuppression-related lymphoproliferative disorders continues to evolve. An increasing number of patients are living with altered immune status due to HIV, solid organ or hematopoietic stem cell transplant, treatment of autoimmune disease, or advanced age. This review covers advances in understanding, emerging trends, and revisions to diagnostic guidelines.Recent FindingsThe tumor microenvironment, including interactions between the host immune system and tumor cells, is of increasing interest in the setting of immunosuppression. While...
Source: Current Hematologic Malignancy Reports - June 12, 2020 Category: Hematology Source Type: research

Choosing Wisely ® in Hematology: Have We Made a Difference?
AbstractPurpose of ReviewThe Choosing Wisely® initiative, led by the American Board of Internal Medicine Foundation in collaboration with national professional medical societies, aims to help patients choose care that is essential, free from harm, and evidence-based. The American Society of Hematology has advocated practices specific to hematology for physicians and patients to examine carefully. Here, we summarize various barriers to adopting these practices, interventions used to improve adoption, and challenges in measuring the effectiveness of these interventions.Recent FindingsThe Choosing Wisely ® campaign has beco...
Source: Current Hematologic Malignancy Reports - June 11, 2020 Category: Hematology Source Type: research

Hematopoietic Cell Transplantation and Adoptive Cell Therapy in Peripheral T Cell Lymphoma
AbstractPurpose of ReviewPeripheral T cell lymphomas (PTCLs) are a heterogeneous group of diseases and represent approximately 10 –15% of all non-Hodgkin lymphomas. Multiagent chemotherapy with a CHOP (cyclophosphamide, adriamycin, vincristine, prednisone)-like regimen is the current standard of care in the frontline setting, but outcomes for PTCL patients generally remain poor. Strategies used to improve survival and reduce the risk of relapse in PTCL patients include autologous hematopoietic cell transplant (autoHCT) and allogeneic HCT (alloHCT). Due to the relative rarity of these diseases, the evidence supporting the...
Source: Current Hematologic Malignancy Reports - June 10, 2020 Category: Hematology Source Type: research

The Evolution of Targeted Therapies in Chronic Lymphocytic Leukaemia
AbstractPurpose of ReviewThe treatment landscape for chronic lymphocytic leukaemia (CLL) is rapidly evolving, with several targeted agents recently approved. These compounds have dramatically changed the natural history of the disease.Recent FindingsHowever, with the array of effective therapies commercially available, the challenge is to define tailored treatment strategies able to realize a balance between treatment efficacy and toxicity or tolerance. New algorithms of treatment are being developed, and it appears that minimal residual disease (MRD) directed therapy will become the norm in the future.SummaryClinical tria...
Source: Current Hematologic Malignancy Reports - June 3, 2020 Category: Hematology Source Type: research

NPM1 Biology in Myeloid Neoplasia
AbstractPurpose of ReviewNucleophosmin (NPM1) mutations are encountered in myeloid neoplasia and are present in ~  30% of de novo acute myeloid leukemia cases. This review summarizes features of mutantNPM1-related disease, with a particular emphasis on recent discoveries relevant to disease monitoring, prognostication, and therapeutic intervention.Recent FindingsRecent studies have shown thatHOX/MEIS gene overexpression is central to the survival ofNPM1-mutated cells. Two distinct classes of small molecule drugs, BH3 mimetics and menin-MLL interaction inhibitors, have demonstrated exquisite leukemic cell toxicity in prec...
Source: Current Hematologic Malignancy Reports - June 2, 2020 Category: Hematology Source Type: research

Novel Therapies in the Treatment of Adult Acute Lymphoblastic Leukemia
AbstractPurpose of ReviewAcute lymphoblastic leukemia (ALL) is a rare hematologic malignancy. Advances in multi-agent chemotherapy have resulted in dramatic improvements in the number of pediatric cases that result in a cure; however, until recently, treatment options for older adults or patients with relapsed and refractory disease were extremely limited. This review seeks to describe in greater detail a number of emerging novel treatment modalities recently approved for this cancer.Recent FindingsIn this review, we discuss a number of recently approved novel therapies for ALL, including new approaches with targeted tyros...
Source: Current Hematologic Malignancy Reports - May 21, 2020 Category: Hematology Source Type: research

Primary Cutaneous CD30+ Lymphoproliferative Disorders: a Comprehensive Review
AbstractPurpose of ReviewPrimary cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPDs) are the second most common cutaneous lymphomas after mycosis fungoides and Sezary syndrome. They include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP), and borderline lesions. The purpose of this literature review is to consolidate the available evidence on the primary cutaneous CD30+ LPD in order to define the tools for correct diagnosis and appropriate treatment.Recent FindingsThe current body of knowledge regarding the clinical features, histopathologic changes, recently described ...
Source: Current Hematologic Malignancy Reports - May 19, 2020 Category: Hematology Source Type: research

Radiogenomics and Its Role in Lymphoma
AbstractPurpose of ReviewImaging features of lymphoma vary regionally. Awareness of site-specific key imaging characteristics of lymphoma can aid in rapid staging and assist in prompt treatment. FDG PET/CT and conventional MRI are readily available diagnostic modalities with excellent sensitivity and good specificity. Diagnostic specificity can be enhanced using emerging PET radiotracers, e.g., FLT and FET.Recent FindingsEmerging research has shown higher dimensional analysis (radiomics and radiogenomics) of imaging data can yield information of the underlying genetic aberrations in lymphoma, which can aid in assessing rea...
Source: Current Hematologic Malignancy Reports - May 19, 2020 Category: Hematology Source Type: research

Management of Ibrutinib Toxicities: a Practical Guide
AbstractPurpose of ReviewIbrutinib is a first-in-class, highly potent Bruton tyrosine kinase inhibitor which has become standard of care for patients with chronic lymphocytic leukaemia and other lymphoproliferative disorders. It requires indefinite administration which places emphasis on toxicity and long-term tolerance.Recent FindingsExtensive use of ibrutinib in studies and clinical practice has better defined its full toxicity profile which has made its use more challenging than initially foreseen. In particular, dysrhythmias, bleeding, infections and constitutional symptoms have been reported and can result in dose red...
Source: Current Hematologic Malignancy Reports - May 14, 2020 Category: Hematology Source Type: research