Initial and Consolidation Therapy for Younger Patients with Mantle Cell Lymphoma
Mantle cell lymphoma is an incurable B-cell malignancy. Treatment of young fit patients is particularly challenging, because careful consideration should be made when building a long-term treatment strategy that would provide longer remissions and increase patients ’ quality of life. Most young fit patients achieve long remissions with a combination of immunochemotherapy containing rituximab and high-dose cytarabine, followed by high-dose chemotherapy and autologous stem-cell transplantation. The addition of maintenance therapy with rituximab following autol ogous stem-cell transplantation prolongs the time to relaps...
Source: Hematology/Oncology Clinics of North America - August 4, 2020 Category: Cancer & Oncology Authors: Daniel Guy, Brad S. Kahl Source Type: research

Cell Cycle Dysregulation in Mantle Cell Lymphoma
This article reviews aberrations of cell cycle genes in MCL cells and clinical trials of CDK4/6 inhibitors for MCL. Integrative longitudinal functional genomics is discussed as a strategy to discover genomic drivers for resistance in cancer cells and cancer-immune interactions that potentially contribute to the clinical response to palbociclib combination therapy in MCL. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - August 1, 2020 Category: Cancer & Oncology Authors: Kevin Wang, Xiangao Huang, Maurizio Di Liberto, Selina Chen-Kiang Source Type: research

Current Role and Emerging Evidence for Bruton Tyrosine Kinase Inhibitors in the Treatment of Mantle Cell Lymphoma
The Bruton tyrosine kinase inhibitors (BTKi), acalabrutinib, ibrutinib, and zanubrutinib, are all approved in the United States for the treatment of relapsed mantle cell lymphoma (MCL). BTKi as a class have become the preferred therapy for most of the patients with relapsed MCL, and ongoing clinical trials are evaluating whether combining BTKi with other targeted agents may deepen response and further improve outcomes. Emerging evidence supports the efficacy of BTKi-containing combinations as frontline treatment, and clinical studies to define the role of this class of drugs for newly diagnosed patients with MCL are in pro...
Source: Hematology/Oncology Clinics of North America - August 1, 2020 Category: Cancer & Oncology Authors: David A. Bond, Kami J. Maddocks Source Type: research

Allogeneic Transplantation and Chimeric Antigen Receptor-Engineered T-Cell Therapy for Relapsed or Refractory Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) accounts for fewer than 10% of non-Hodgkin lymphoma. There is a high initial response rate to chemotherapy and rituximab, but a nearly universal risk of relapse. Allogeneic hematopoietic cell transplantation (allo-HCT) provides one of the only curative options. We review the role of allo-HCT for relapsed and refractory (R/R) MCL and discuss a novel promising approach using autologous chimeric antigen receptor-engineered T (CAR-T) cells. We review preliminary safety and efficacy data of 2 pivotal trials investigating the use of CD19-targeted CAR-T cells for R/R MCL after ibrutinib failure and disc...
Source: Hematology/Oncology Clinics of North America - August 1, 2020 Category: Cancer & Oncology Authors: Jordan Gauthier, David G. Maloney Source Type: research

Mantle Cell Lymphoma: Biologic Insights to Bedside Impact
Since the recognition of mantle cell lymphoma (MCL) as a distinct entity within B-cell non-Hodgkin lymphoma, progress in the evolution of treatment has occurred at a pace perhaps more rapid than any other lymphoma subtype. Better diagnostic and prognostic tools extending from cytogenetics, immunohistochemistry (SOX11, Ki-67), and assessment of p53 status have augmented clinical assessments (such as the Mantle Cell International Prognostic Index) to define heterogeneity among MCL patients. While chemotherapy-based treatment has long been a mainstay of management, novel agents targeting the cell cycle, Bruton's Tyrosine Kina...
Source: Hematology/Oncology Clinics of North America - July 30, 2020 Category: Cancer & Oncology Authors: John P. Leonard Tags: Preface Source Type: research

Watch and Wait in Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) is a biologically heterogeneous disease, and patients may experience a clinical course ranging from indolent to very aggressive. Observational studies suggest that a subset of patients can be safely observed for a period of months to years from initial diagnosis without adversely impacting their outcomes. However, identification of candidates for the “watch and wait” approach remains challenging because selection criteria are not well-defined. Studies that prospectively stratify patients on the basis of MCL biology and disease risk will be informative, and patients with indolent MCL m...
Source: Hematology/Oncology Clinics of North America - July 29, 2020 Category: Cancer & Oncology Authors: Christina Lee, Peter Martin Source Type: research

Is Limited-Stage Mantle Cell Lymphoma Curable and How Is It Best Managed?
This article reviews literature pertaining to management of patients with limited-stage MCL and discusses approach to treatment. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - July 29, 2020 Category: Cancer & Oncology Authors: Jason T. Romancik, Jonathon B. Cohen Source Type: research

What Is Responsible for Heterogeneity in Mantle Cell Lymphoma Biology and Outcomes?
Mantle cell lymphoma, despite its common derivation from a t(11;14) error that occurs in a na ïve B-cell leading to overexpression of cyclin D1 protein, is characterized by substantial heterogeneity in biology and clinical outcome. Unlike other non-Hodgkin lymphoma types, it is more common in men. Clinical presentation patterns vary from nodal to splenomegaly with leukemia to gastrointestin al involvement. Biological variability is linked to tumor cell proliferation. Increased monocyte/macrophages and their associated proinflammatory cytokines are associated with inferior outcomes. These clues mandate that new treatme...
Source: Hematology/Oncology Clinics of North America - July 26, 2020 Category: Cancer & Oncology Authors: Thomas E. Witzig Source Type: research

Molecular Pathogenesis of Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) is a mature B-cell neoplasm with heterogeneous clinical behavior molecularly characterized by the constitutive overexpression of cyclin D1 and deregulation of different signaling pathways. SOX11 expression determines an aggressive phenotype associated with accumulation of many chromosomal alterations and somatic gene mutations. A subset of patients with the SOX11-negative leukemic non-nodal MCL subtype follows an initial indolent clinical evolution and may not require treatment at diagnosis, although eventually may progress to an aggressive disease. We discuss the genetic and molecular alteration...
Source: Hematology/Oncology Clinics of North America - July 22, 2020 Category: Cancer & Oncology Authors: Alba Navarro, S ílvia Beà, Pedro Jares, Elías Campo Source Type: research

Follicular Lymphoma
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - June 23, 2020 Category: Cancer & Oncology Authors: Jonathan W. Friedberg Source Type: research

Copyright
ELSEVIER (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - June 23, 2020 Category: Cancer & Oncology Source Type: research

Contributors
GEORGE P. CANELLOS, MD (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - June 23, 2020 Category: Cancer & Oncology Source Type: research

Contents
Jonathan W. Friedberg (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - June 23, 2020 Category: Cancer & Oncology Source Type: research

Hematology/Oncology Clinics of North America
Mantle Cell Lymphoma (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - June 23, 2020 Category: Cancer & Oncology Source Type: research

Toward a Cure for Follicular Lymphoma
In 1984, Horning and Rosenberg published a seminal paper describing the natural history of follicular lymphoma (then also referred to as nodular poorly differentiated lymphocytic lymphoma) emphasizing the incidence of spontaneous regressions and histological transformation over time, the lack of benefit of early therapeutic intervention, and the incurable nature of the disease with a median overall survival of 11 years. They concluded that “an increased understanding of the cause and regulation of these low-grade B-cell neoplasms and their potential manipulation by new therapeutic approaches offers exciting avenues f...
Source: Hematology/Oncology Clinics of North America - May 20, 2020 Category: Cancer & Oncology Authors: Jonathan W. Friedberg Tags: Preface Source Type: research

Clinical and Biological Prognostic Factors in Follicular Lymphoma
Follicular lymphoma comprises approximately 20-30% of all cases of B-cell lymphomas. Median survival has improved significantly in the modern era. Prognostic factors include histologic grade, cytogenetics, molecular mutations, the tumor microenvironment, and tumor burden. Clinical prognostic indices are available and increasingly incorporate genetic information. Prognostic factors also arise during the course of treatment. Early progression within 24 months of initial chemoimmunotherapy is an adverse prognostic marker of inferior survival. Other high-risk populations include those with double refractory disease or those wi...
Source: Hematology/Oncology Clinics of North America - May 6, 2020 Category: Cancer & Oncology Authors: Anand A. Patel, Sonali M. Smith Source Type: research

Epidemiology of Follicular Lymphoma
Follicular lymphoma (FL) is a common indolent lymphoma subtype in Western countries. FL incidence increases with age, and shows considerable variation by race/ethnicity and geography. In the United States and France, FL incidence has been stable since 2000, whereas in other Western and Asian countries it has been increasing. Five-year relative survival rates have been increasing in Western and Asian countries. Progress on identifying FL-specific risk factors has accelerated with the implementation of the InterLymph nested classification and the availability of larger epidemiologic studies and pooled analyses. Identificatio...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: James R. Cerhan Source Type: research

Initial Treatment of Early Stage and Low Tumor Burden Follicular Lymphoma
Patients with early stage follicular lymphoma frequently have prolonged overall survival and 40% may remain progression-free 20  years after receiving radiation therapy alone. Thus, such an approach is often considered in this population. Patients with advanced-stage disease but low tumor burden do not achieve a survival benefit by initiation treatment but early therapy with rituximab can improve quality of life and prolong time until need for further treatment and/or chemotherapy. Patients with advanced-stage follicular lymphoma who have low tumor burden should be managed in a personalized fashion taking into account...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Jonathon B. Cohen, Brad S. Kahl Source Type: research

Initial Treatment of High Tumor Burden Follicular Lymphoma
Follicular lymphoma is the most common indolent B-cell lymphoma, with most patients presenting with advanced-stage disease. Patients who are symptomatic or otherwise meet the criteria for high tumor burden merit immediate intervention with combination immunochemotherapy, although more recently, “chemotherapy-free” approaches including novel therapies have shown benefit in this treatment-naïve population. In this review, the authors critically appraise the criteria for high tumor burden, the various options available for initial therapy, and the optimal post-treatment surveillance stra tegies. (Source: Hema...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Ciara L. Freeman, Laurie H. Sehn Source Type: research

Antibody Therapy Maintenance in Follicular Lymphoma
Because patients with follicular lymphoma (FL) usually experience repeated disease recurrences, maintenance treatment is an attractive option to prolong remission after induction therapy. Rituximab maintenance therapy has been shown in multiple randomized studies to significantly improve progression-free survival in FL with both low and high tumor burden after induction therapy, independently of patient and disease characteristics. Several questions regarding the use of antibody directed against CD20 (anti-CD20) maintenance remain open, including the optimal antibody administration schedule and duration, the risk/benefit r...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Camille Golfier, Gilles Salles Source Type: research

Early Progression of Follicular Lymphoma
Follicular lymphoma is the most common subtype of indolent non-Hodgkin lymphoma. Although a majority of patients have a favorable prognosis, a subset of patients experiences early treatment failure. Progression of disease within 24 months of initial chemoimmunotherapy is associated with inferior survival. The biology of early progression is the subject of ongoing study and depends on the unique genetic composition of neoplastic cells and their interaction with a complex tumor microenvironment. Clinicogenetic prognostic indices have been developed to identify high-risk patients. Several have been validated but are limited i...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Jodi J. Lipof, Paul M. Barr Source Type: research

The Biological Basis of Histologic Transformation
Histologic transformation of follicular lymphoma remains the leading cause of follicular lymphoma-related mortality in the rituximab era. Both the diverse timing of transformation and heterogeneity in associated genomic events suggest that histologic transformation may itself comprise distinct disease entities. Successive indolent and transformation episodes occur by divergent clonal evolution from an inferred common progenitor cell, representing a potential therapeutic target. Existing biological knowledge largely pre-dates anti-CD20 therapy, and further prospective validation is essential. Inclusion of transformation cas...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Emil A. Kumar, Jessica Okosun, Jude Fitzgibbon Source Type: research

Treatment of Histologic Transformation
Histologic transformation from follicular lymphoma to aggressive lymphoma historically had a poor prognosis. Routine use of anti-CD20 antibody rituximab has changed the landscape of follicular lymphoma (FL) such that outcomes are improved in select patients, similar to de-novo diffuse large B-cell lymphoma. Several biological and clinical biomarkers can predict risk of transformation, and ongoing research is improving understanding of the biology surrounding the transformation process. This review provides an overview of risk factors, prognosis, and treatment of histologic transformation of FL. (Source: Hematology/Oncology...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Carla Casulo Source Type: research

Novel Agents Beyond Immunomodulatory Agents and Phosphoinositide-3-Kinase for Follicular Lymphoma
Follicular lymphoma is the most common indolent non-Hodgkin lymphoma. Although median overall survival rates exceed 12  years with rituximab, follicular lymphoma remains largely incurable. The growing understanding of the molecular drivers of lymphomagenesis and the tumor microenvironment have led to novel therapies. Prognostic markers have identified a subset of patients with chemoresistant and/or refractory disea se-associated poor outcomes. We identify the patients with follicular lymphoma in need of novel therapies, describe the drivers of lymphomagenesis and importance of the tumor microenvironment, and summarize...
Source: Hematology/Oncology Clinics of North America - May 5, 2020 Category: Cancer & Oncology Authors: Collin K. Chin, Loretta J. Nastoupil Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 24, 2020 Category: Cancer & Oncology Authors: Andrew A. Lane Source Type: research

Copyright
ELSEVIER (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 24, 2020 Category: Cancer & Oncology Source Type: research

Contributors
GEORGE P. CANELLOS, MD (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 24, 2020 Category: Cancer & Oncology Source Type: research

Contents
Andrew A. Lane (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 24, 2020 Category: Cancer & Oncology Source Type: research

Forthcoming Issues
Follicular Lymphoma (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 24, 2020 Category: Cancer & Oncology Source Type: research

Cellular Therapy in Follicular Lymphoma
A subset of follicular lymphoma patients with high-risk clinical features continues to pose a therapeutic challenge. Hematopoietic stem cell transplantation is a suitable consolidative treatment option for these patients. Data on chimeric antigen receptor T-cell therapy are promising in relapsed/refractory and transformed patients. The increasing armamentarium of nontransplant options coupled with the associated potential long-term sequelae of transplantation raises questions about the placement of transplant strategies in the follicular lymphoma treatment hierarchy. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 16, 2020 Category: Cancer & Oncology Authors: Jessica Okosun, Silvia Montoto Source Type: research

Phosphatidylinositol-3-Kinase Inhibition in Follicular Lymphoma
We describe the efficacy and toxicities of phosphatidylinositol-3-kinase inhibitors. Response rates in highly refractory disease are high, demonstrate few long-term remissions, and have high long-term toxicity. Early data on dosing and combination strategies are promising and may change how we use these agents in the coming years. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 9, 2020 Category: Cancer & Oncology Authors: Ryan C. Lynch, Ajay K. Gopal Source Type: research

Immunomodulatory Agents in Follicular Lymphoma
New treatment strategies in follicular lymphoma (FL) are driven by a deeper understanding of microenvironmental cues supporting lymphomagenesis, chemoresistance, and immuno-escape. These immune-mediated signaling pathways contribute to initial learnings and clinical successes with lenalidomide, the first, oral, non-chemotherapeutic immunomodulatory drug, combined with anti-CD20 antibodies. This combination of lenalidomide with rituximab showed similar efficacy to chemoimmunotherapy (CIT) in first-line patients requiring therapy, and is approved in relapsed/refractory FL. We review the biology supporting the rationale for a...
Source: Hematology/Oncology Clinics of North America - April 8, 2020 Category: Cancer & Oncology Authors: Loic Ysebaert, Franck Morschhauser Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) has to be considered an orphan tumoral disease. BPDCN is a good model concerning the structuring and the organization of a concerted medical program on a nation-based, transnational, or international level. In 2019 in France the diagnosis process for BPDCN was clearly established. Two prospective clinical trials are ongoing. Because of the difficulties in diagnostic procedures and the rarity of the disease it is important that European countries collaborate to build a real European network to ensure the best and equitable medical care to all BPDCN patients. (Source: Hema...
Source: Hematology/Oncology Clinics of North America - April 3, 2020 Category: Cancer & Oncology Authors: Eric Deconinck Source Type: research

Immunotherapies Targeting CD123 for Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, difficult-to-diagnose, highly aggressive myeloid malignancy with poor prognosis and no standard of care. The interleukin-3 receptor α, or CD123, is highly expressed in patients with myeloid malignancies, particularly acute myeloid leukemia and BPDCN. CD123 is overexpressed on leukemic stem cells compared with normal hematopoietic stem cells, suggesting CD123 as an attractive immunotherapeutic target. To date, multiple CD123-tar geted therapeutic avenues have been explored to treat BPDCN and other CD123+ hematologic malignancies. This review summarizes im...
Source: Hematology/Oncology Clinics of North America - March 27, 2020 Category: Cancer & Oncology Authors: Tongyuan Xue, L. Elizabeth Budde Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm
Clinical and biological presentation of patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN) is depicted to highlight criteria that might alert physicians. Diagnosis of BPDCN is still challenging and requires (1) immunophenotyping of blood or bone marrow aspiration using several markers (CD4, CD56, HLA-DR, myeloid and lymphoid lineage markers) and should include pDC markers such as CD123, cTCL1, CD303, and CD304, and/or (2) pathologic analysis of cutaneous lesions, also with immunohistochemistry using markers specific to BPDCN. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 23, 2020 Category: Cancer & Oncology Authors: Eric Deconinck, Tony Petrella, Francine Garnache Ottou Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive malignancy derived from the plasmacytoid dendritic cell that commonly involves the skin. Cutaneous involvement is often the initial presentation, with deep purple or red-brown macules, plaques, or tumors. As such, dermatologists may be the first to see these patients and, in addition to oncologists, should be familiar with its presentation to facilitate early diagnosis, helping to distinguish it from acute myelogenous leukemia cutis. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 20, 2020 Category: Cancer & Oncology Authors: Jesse P. Hirner, John T. O ’Malley, Nicole R. LeBoeuf Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm in 2020 and Beyond
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive blood cancer with poor patient outcomes. Relatively little was known about the pathobiology and therapeutic sensitivities of the disease until recently. Consequently, the approach to the patient was uninformed, empiric, and inadequate. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 20, 2020 Category: Cancer & Oncology Authors: Andrew A. Lane Tags: Preface Source Type: research

CD123 as a Therapeutic Target Against Malignant Stem Cells
Hematopoiesis is a tightly regulated process that originates from highly specialized cells, hematopoietic stem cells (HSCs). Many cancers can arise and be maintained by malignant stem cells. In acute myeloid leukemia, leukemic stem cells (LSCs) are identified by their immunophenotype, which is partly shared with normal HSCs (CD34+CD38 −). However, LSCs also possess unique immunophenotypic features that can be used to distinguish them from HSCs and therapeutically target them. One such unique immunophenotypic marker is CD123, found to be aberrantly expressed in leukemic stem, progenitor, and blast cells. Thus, CD123 i...
Source: Hematology/Oncology Clinics of North America - March 19, 2020 Category: Cancer & Oncology Authors: Mayumi Sugita, Monica L. Guzman Source Type: research

Cytogenetics of Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a skin-tropic hematopoietic malignancy. Approximately 60% of cases with analyzable karyotyping results show complex karyotypes. Losses are more frequently found than copy-number gains. Recurrently deleted regions include tumor suppressor genes. No specific chromosomal abnormalities have been demonstrated in BPDCN, but genomic rearrangements involving the MYB family genes and MYC were identified. One-third of cases of BPDCN harbor the 8q24 rearrangement, most frequently with 6p21 harboring RUNX2, which is associated with immunoblastoid cytomorphology and MYC expression...
Source: Hematology/Oncology Clinics of North America - March 18, 2020 Category: Cancer & Oncology Authors: Kana Sakamoto, Kengo Takeuchi Source Type: research

Tagraxofusp for Blastic Plasmacytoid Dendritic Cell Neoplasm
Tagraxofusp, a CD123-targeted immunotoxin, is the first FDA-approved treatment for patients 2 years and older with blastic plasmacytoid dendritic cell neoplasm (BPDCN). It has been shown to be safe and effective in treatment-na ïve and previously treated adult patients, with high rates of successful bridging to hematopoietic stem cell transplantation. The pediatric experience is more limited but demonstrates safety. Given the risk of potentially fatal capillary leak syndrome with tagraxofusp, judicious patient selection i s recommended. Combination therapy with hypomethylating agents and/or BCL-2 inhibitors are ration...
Source: Hematology/Oncology Clinics of North America - March 18, 2020 Category: Cancer & Oncology Authors: Danielle Hammond, Naveen Pemmaraju Source Type: research

Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm
This article outlines novel targeted approaches that are in preclinical or clinical development for BPDCN. Although there is no known targetable genetic abnormality that defines BPDCN, data from functional testing of primary tumors, gene expression analyses, and adaptation of targeted drug approaches from other cancers to BPDCNs harboring specific mutations have nominated several promising strategies. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 18, 2020 Category: Cancer & Oncology Authors: Andrew A. Lane Source Type: research

Chemotherapy Options for Blastic Plasmacytoid Dendritic Cell Neoplasm
BPDCN is ultimately a bone marrow disease requiring induction-type eradication therapy followed by hematopoietic stem cell transplant (HSCT) to achieve long-lasting remissions or cure. Various regimens have been applied to this disease with varying success. A cumulative review of the literature suggests more intense regimens have greater efficacy with acute lymphoblastic leukemia regimens preferred to acute myeloid leukemia regimens. This approach benefits fit patients who are eligible for HSCT; however, most BPDCN patients require other treatment options. The recent FDA approval of the CD123-targeted agent tagraxofusp pro...
Source: Hematology/Oncology Clinics of North America - March 18, 2020 Category: Cancer & Oncology Authors: Michael Haddadin, Justin Taylor Source Type: research

Hematopoietic Cell Transplant for Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy derived from precursors of plasmacytoid dendritic cells. Historically, BPDCN has had few available treatment options and a poor prognosis. The emergence of novel targeted therapies, namely tagraxofusp, has changed the treatment landscape of BPDCN, but data are lacking regarding the long-term durability of responses. Despite absence of randomized data, allogeneic hematopoietic cell transplant has become the de facto option for patients with BPDCN who achieve a first complete remission. As new therapies continue to emerge, it wil...
Source: Hematology/Oncology Clinics of North America - March 17, 2020 Category: Cancer & Oncology Authors: Mohamed A. Kharfan-Dabaja, Mohamad Cherry Source Type: research

Blastic Plasmacytoid Dendritic Cell Neoplasm in Children
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematological malignancy, derived from plasmacytoid dendritic cells. It mainly occurs in older adults, but has been reported across all age groups. Most patients present with skin lesions with or without marrow involvement and leukemic dissemination. Treatment with high-risk acute lymphoblastic leukemia therapy regimens with central nervous system prophylaxis is recommended in pediatric patients. Stem cell transplant in children is recommended for relapsed/refractory disease or high-risk disease at presentation. New targeted therapies including the ...
Source: Hematology/Oncology Clinics of North America - March 9, 2020 Category: Cancer & Oncology Authors: Yixian Li, Victoria Sun, Weili Sun, Anna Pawlowska Source Type: research

Molecular Features of Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm with a dismal prognosis and no standard therapy. In the past, its cellular ontogenesis was obscure, and BPDCN had been erroneously named CD56+/TdT+  blastic NK cell tumor and CD4+/CD56+ hematodermic neoplasm. Finally, in 2008, the BPDCN was correctly recognized as a neoplasm deriving from the malignant transformation of plasmacytoid dendritic cell precursors and classified among the myeloid neoplasms. Since then, the understanding of BPDCN bio logy has improved rapidly: the DNA mutational status of BPDCN has been extensively investigat...
Source: Hematology/Oncology Clinics of North America - March 4, 2020 Category: Cancer & Oncology Authors: Maria Rosaria Sapienza, Stefano Pileri Source Type: research

Erratum
For the article on “Radiation Therapy for Prostate Cancer” in the February 2020 issue of Hematology/Oncology Clinics of North America (Volume 34, Issue 1), a few errors occurred regarding the mentions of the Tables. On page 48, a mention of Table 1 was erroneously placed in the section, “Intermediate-Risk Disea se”. On page 51, a mention of Table 1 was erroneously placed in the section, “Definition and historical perspective”. Also on page 51, at the end of the section, “Definition and historical perspective”, the mention of Table 6 was incorrectly labeled as T...
Source: Hematology/Oncology Clinics of North America - February 21, 2020 Category: Cancer & Oncology Source Type: research

Myelodysplastic Syndromes
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - February 21, 2020 Category: Cancer & Oncology Authors: David P. Steensma Source Type: research

Copyright
ELSEVIER (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - February 21, 2020 Category: Cancer & Oncology Source Type: research

Contributors
GEORGE P. CANELLOS, MD (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - February 21, 2020 Category: Cancer & Oncology Source Type: research

Contents
Erratumxiii (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - February 21, 2020 Category: Cancer & Oncology Source Type: research