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NCCN Annual Congress Covers Treatment Advancements in Hematologic Malignancies; New NCCN Guidelines Presented

By Rashmi Kumar, PhD, Senior Manager, Clinical Content, NCCN; Courtney Smith, PhD, Oncology Scientist/Medical Writer, NCCN; and Hema Sundar, PhD, Oncology Scientist/Senior Medical Writer, NCCN

On Friday, September 30 and Saturday, October 1, 2016, the National Comprehensive Cancer Network® (NCCN®) hosted its 11th Annual Congress: Hematologic Malignancies™ in New York, New York.

The congress was moderated by Andrew D. Zelenetz, MD, PhD, Memorial Sloan Kettering Cancer Center, Panel Chair of the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Non-Hodgkin’s Lymphomas (NHL), and Ranjana Advani, MD, Stanford Cancer Institute, member of the NCCN Guidelines® Panel for NHL. More than 520 oncology professionals attended the event, which featured expert speakers from the NCCN Member Institutions.

The presentations focused on a range of pertinent topics in hematologic malignancies, including myeloproliferative neoplasms (MPN), acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), chronic myelogenous leukemia (CML), chronic lymphocytic leukemia (CLL), T-cell and B-cell lymphomas, multiple myeloma (MM), and supportive care issues in hematologic malignancies. In addition, numerous challenging and/or rare patient-cases were presented in three interactive panel discussions.

Friday’s session commenced with opening remarks from Dr. Zelenetz followed by presentation of the new NCCN Guidelines for MPN by Ruben A. Mesa, MD, Mayo Clinic Cancer Center, NCCN Guidelines Panel Chair for MPN.  The new Guidelines provide recommendations for the diagnosis, risk stratification, treatment, and supportive care strategies for myelofibrosis (MF). Dr. Mesa discussed the prognostic scoring systems used for the risk stratification, assessment of symptom burden, JAK2 inhibitor therapy with ruxolitinib, and management of MF-associated anemia. He highlighted some of the emerging treatment options for MF, including the JAK2/FLT3 inhibitor, pacritinib and the JAK1/2 inhibitor, momelotinib, which are being evaluated in ongoing clinical trials.

Jessica K. Altman, MD, Robert H. Lurie Comprehensive Cancer Center of Northwestern University, discussed the role of novel therapies in the treatment of AML. Foremost, Dr. Altman highlighted the need to better define “AML” due to the heterogeneity of the disease and illustrated the diversity within the disease with a comparison of two patient case studies. Novel agents and the role of mutations were reviewed. A lunch symposium on Saturday presented by Dr. Altman and Richard M. Stone, MD, Dana-Farber Cancer Institute, elaborated on the future of targeted therapies for AML, focusing on FLT3 inhibitors, the antibody-drug conjugates gemtuzumab and inotuzumab, BCL-2 inhibitors, and IDH1/2 inhibitors.

Jerald P. Radich, MD, Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance, NCCN Guidelines Panel Chair for CML, talked about the impact of generic imatinib on the treatment of chronic phase CML. While generic imatinib is cost-effective and good for long-term use, the second generation tyrosine kinase inhibitors (TKI) are better at inducing deep remissions and preventing disease progression to advanced phase, Dr. Radich explained. The selection of a front-line TKI should be based on the risk stratification, the treatment goal (of preventing disease progression and achieving complete molecular response), and patient’s comorbidities, he pointed out. He emphasized that compliance to TKI therapy is essential to achieve treatment goals.

The session on Friday concluded with the first series of patient case studies and panel discussion presented by Dr. Altman, Dr. Mesa, and Dr. Radich. Dr. Altman opened the discussion on the treatment of elderly patients with AML. Emphasis was placed on the availability of several treatment options for these patients including intensive treatments, transplant, clofarabine, hypomethylating agents, and novel treatment approaches. In addition to comorbid conditions, molecular studies were recommended to guide treatment options and advocated for consideration of more intensive therapy for fit patients with a caveat that for patients with a low probability of benefit from intensive treatment despite fitness, less intensive therapy or an investigational approach should be pursued. Dr. Radich discussed the management of a patient with newly diagnosed CML, highlighting the importance of monitoring response to treatment, evaluating patient compliance, and mutational analysis prior to changing treatment when response milestones are not achieved.  Dr. Mesa described the role of JAK2 mutation testing in patients with clonal hematopoiesis of indeterminate potential (CHIP) and the management of JAK2 mutated splanchnic vein thrombosis.

Saturday morning opened with patient case studies and panel discussion presented by Dr. Advani; Steven M. Horwitz, MD, Memorial Sloan Kettering Cancer Center; and Francine Foss, MD, Yale Cancer Center/Smilow Cancer Hospital. The panelists focused on the diagnosis, workup, and treatment of three rare subtypes of T-cell lymphoma: extranodal NK/T-cell lymphoma, adult T-cell leukemia/lymphoma, and primary cutaneous gamma delta T-cell lymphoma. 

The next two presentations focused on the treatment of peripheral T-cell lymphomas (PTCL) and supportive care in the management of cutaneous T-cell lymphomas (CTCL). Dr. Horwitz reviewed the current and emerging treatment options for newly diagnosed as well as relapsed PTCL, highlighting some of the ongoing clinical trials evaluating CHOP-like chemotherapy in combination with novel agents to improve clinical outcomes in different subtypes of newly diagnosed PTCL. JAK/STAT signalling pathways and IDH2 mutations are also being considered as novel targets in various PTCL subtypes. DUSP22 and TP63 rearrangements are emerging as prognostic indicators of outcome in ALK-negative anaplastic large cell lymphoma. Erin Kopp, ACNP-BC, City of Hope Comprehensive Cancer Center, provided an overview of multiple options for supportive care for patients with CTCL, emphasizing that symptoms and side effects should be assessed often and thoroughly. Supportive care complements curative care and is critical to maintaining quality of life, Ms. Kopp concluded.

Dr. Zelenetz discussed the methods of determining cell of origin (COO) and its prognostic significance in diffuse large B-cell lymphoma (DLBCL). COO identifies two distinct subtypes of DLBCL: germinal center B-cell (GCB) and activated B-cell (ABC). Immunohistochemistry remains inexact and gene expression profiling of formalin-fixed paraffin-embedded tissue is emerging as a superior clinical assay for the determination of COO, Dr. Zelenetz noted. While treatment options appear to be influenced by COO (e.g., lenalidomide and ibrutinib have activity in ABC-DLBCL) the prognostic significance of COO remains controversial. Although an important consideration, at the present time, COO should not be used as the only factor to influence treatment selection, Dr. Zelenetz acknowledged. 

Dr. Advani summarized the data from clinical trials that have evaluated the early PET scan-guided treatment approach for early stage classical Hodgkin’s lymphoma (CHL) and outlined potential strategies to optimize therapy. Chemotherapy alone would be appropriate for female patients less than 35 years old with axillary and mediastinal involvement whereas combined modality therapy could be considered for patients with favorable disease, especially when it is possible to limit the duration of chemotherapy, Dr. Advani explained. She concluded saying that it is critical to use the Deauville criteria (5-point scale) for the interpretation of interim PET scans, especially when a response-adapted approach is being used for guiding treatment.

Thomas LeBlanc, MD, MA, Duke Cancer Institute, discussed the goals of shared-decision making when treating patients with hematologic malignancies. Using patient scenarios as examples he shared tips on implementing this in clinical practice. Dr. LeBlanc explained that taking into consideration biology of the disease and risk features are important for disease management; however, understanding patient perspective is equally important to optimize care.  He provided three steps to shared-decision making– introducing choice, describing options, and exploring patient preference– and elaborated on what each step entails. He stressed the importance of using principles of good communication through the clinical encounter with patients. By understanding patient goals and implementing shared–decision making in practice, he said physicians can provide the “right care, at the right place, and at the right time.”

The afternoon session commenced with patient case-based discussions on diagnosis and management of monoclonal gammopathy of undetermined significance (MGUS), smoldering myeloma, and MM with renal failure by panelists George Somlo, MD, City of Hope Comprehensive Cancer Center; Carol Huff, MD, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins; and Ola Landgren MD, PhD, Memorial Sloan Kettering Cancer Center.

The case studies segued to three talks encompassing overall management of patients with MM from diagnosis and primary treatment to use of novel agents and optimal supportive care. Dr. Somlo provided an overview on the symptoms, diagnosis, and the continuously evolving treatment of MM. He outlined the revised International Staging System and discussed updated diagnostic and treatment response criteria put forth by the International Myeloma Working Group. He also presented the recently updated NCCN Guidelines for primary therapy and pointed out that autologous stem cell transplant continues to play an important role in consolidating and deepening response to initial treatment. The current treatment paradigm for transplant eligible patients consists of primary therapy with novel drugs followed by autologous stem cell transplant and maintenance therapy. He explained how new agents have improved survival of patients ineligible for transplant and briefly outlined strategies for anticipating, preventing, and managing treatment-related adverse effects including dose-modifying strategies.

Subsequently, Dr. Huff discussed the strategies for integration of the new drugs into the treatment plan of MM. She pointed out that the rapid additions of novel drugs to the treatment armamentarium over the past 12 years have contributed to dramatic improvement in survival rates of patients with MM. In 2015 alone, the FDA approved four new drugs- panobinostat, daratumumab, ixazomib, and elotuzumab for treatment of relapsed/refractory MM. Dr. Huff talked about the mechanisms of action of these new drugs, discussed the data leading to their approval, and highlighted the rapid incorporation of these drugs in the NCCN Guidelines. In conclusion, Dr. Huff noted that despite this progress, a better understanding of sequencing treatment and patient selection is still needed, especially for management of patients with relapsed/refractory disease.

The session on MM was concluded by Kathleen Colson, RN, BSN, BS, Dana-Farber Cancer Institute, whose talk focused on providing supportive care for patients with MM.  She agreed that the explosion of available therapies is no doubt an important step in improving patient outcomes and pointed out that helping patients overcome toxicities and discomfort from those therapies remains a challenge. She noted that the adverse effects with the newer agents are different from older therapies. With good supportive care practices, she said that the increased survival rates we now see can be accompanied by improved quality of life. In her presentation, she described supportive care measures that need to be taken for clinical manifestation from both myeloma and its treatment including bone-related issues, anemia, infections, and peripheral neuropathy.                             

Rebecca B. Klisovic, MD, The Ohio State University Comprehensive Cancer Center – James Cancer Hospital and Solove Research Institute, engaged attendees with new options for relapsed and refractory ALL. Trial data for monoclonal antibodies and chimeric antigen receptor (CAR) T-cells were presented. Immunotherapies have a unique set of toxicities including cytokine-release syndromes and neurologic toxicities. In general, monoclonal antibodies have been shown to be well tolerated and to produce significant response rates though durability data are not available. CAR-T cells continue to demonstrate impressive early responses; however challenges of tolerability and persistence remain.

Steven Coutre, MD, Stanford Cancer Institute, and William G. Wierda, MD, PhD, The University of Texas MD Anderson Cancer Center, debated on the controversies in the front-line management (continuous TKI therapy vs chemoimmunotherapy) of CLL. Dr. Coutre contended that ibrutinib should be used as front-line therapy not only in older patients but also in younger patients since it is associated with higher rates of progression-free survival. Dr. Wierda asserted that first-line chemoimmunotherapy results in deeper remissions that correlate with long-term progression-free and overall survival in specific subgroups of patients. He also noted that follow-up is short with ibrutinib and long-term side effects are unknown. The results of an ongoing randomized trial of fludarabine, cyclophosphamide, and rituximab compared with ibrutinib plus rituximab will provide definitive answers to this debate in the near future.

The congress concluded with closing remarks from Dr. Zelenetz.

Accredited materials from the congress will be available shortly through the NCCN Continuing Education Portal.

The NCCN 12th Annual Congress: Hematologic Malignancies is scheduled for Friday, October 6, and Saturday, October 7, 2017 at the Hilton San Francisco Union Square in San Francisco, California.