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Highlights from the NCCN 10th Annual Congress: Hematologic MalignanciesTM

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

On Friday, October 16, and Saturday, October 17, 2015, the National Comprehensive Cancer Network® (NCCN®) hosted its 10th Annual Congress: Hematologic Malignancies™.

This year’s event was moderated by Andrew D. Zelenetz, MD, PhD, Memorial Sloan Kettering Cancer Center, NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Panel Chair for Non-Hodgkin’s Lymphomas (NHL), and Dr. Ranjana Advani, MD, Stanford Cancer Institute, Member of the NCCN Guidelines® Panel for NHL. More than 540 oncology professionals attended the event, which featured expert speakers from the NCCN Member Institutions.

The presentations focused on a wide range of topics in hematologic malignancies, including chronic lymphocytic leukemia (CLL), NHL, acute myeloid leukemia (AML), myelodysplastic syndromes (MDS), acute lymphoblastic leukemia (ALL), multiple myeloma (MM), Waldenström’s macroglobulinemia/Lymphoplasmacytic Lymphoma (WM/LPL), Castleman disease, classical Hodgkin lymphoma (CHL), and survivorship issues.  In addition, nine patient case studies were presented in three interactive panel discussions.

Friday’s session commenced with opening remarks from Dr. Zelenetz followed by patient case study presentations involving different subtypes of NHLs and discussion by panelists, Jeremy S. Abramson, MD, Dana-Farber/Brigham and Women’s Cancer Center | Massachusetts General Hospital Cancer Center; Dr. Advani; and Dr. Zelenetz. This interactive session underscored the importance of accurate diagnosis and individualizing treatment options (depending on patient’s age, performance status and comorbidities) in the management of mantle cell lymphoma, ALK-negative anaplastic large cell lymphoma, and double-hit diffuse large B-cell lymphoma.

William G. Wierda, MD, PhD, The University of Texas MD Anderson Cancer Center, summarized data from clinical trials that led to the approval of ibrutinib and idelalisib (small molecule inhibitors of B-cell receptor signalling pathways) for the management of heavily pretreated relapsed/refractory CLL with del (17p) and del (11q). Ibrutinib is also approved for first-line therapy for CLL with del (17p). Venetoclax is an oral Bcl-2 inhibitor with potent monotherapy activity in high-risk relapsed/refractory CLL which may be approved in the near future for the treatment of relapsed/refractory CLL, Dr. Wierda acknowledged.

In the next presentation, Dr. Zelenetz explained the need for new treatment strategies for the management of follicular lymphoma (FL) and provided a comprehensive overview of clinical trials evaluating a variety of emerging treatment options for first-line therapy and relapsed/refractory FL. He also alluded to the new clinicogenomic risk model (M7-FLIPI) that improves risk stratification with the integration of mutational status of seven genes (EP300, FOXO1, CREBBP, CARD11, MEF2B, ARID1A and EZH2), FLIPI and ECOG performance status. M7-FLIPI may help to identify high-risk patients at diagnosis who would be candidates for novel treatments, Dr. Zelenetz noted.

Friday’s session concluded with the presentation on the management of HIV-associated NHLs. Lawrence D. Kaplan, MD, UCSF Helen Diller Family Comprehensive Cancer Center, highlighted the special considerations for the treatment of aggressive NHLs in the patients with HIV-infection. Rituximab should be included with front-line chemotherapy regimens for most patients with the exception of those with CD4 count <50, he pointed out. Antiretroviral therapy, when administered with chemotherapy, achieves better control of HIV replication. Most importantly, communication between the oncologist and HIV-treating physician is critical, Dr. Kaplan emphasized.

Saturday morning opened with three case studies involving myeloproliferative neoplasms (MPNs) and hemophagocytic lymphohistiocytosis (HLH) presented by Peter L. Greenberg, MD, Stanford Cancer Institute; Jessica Altman, MD, Robert H. Lurie Comprehensive Cancer Center of Northwestern University; and Joseph C. Alvarnas, MD, City of Hope Comprehensive Cancer Center. Dr. Greenberg discussed distinctions among classical MPNs, less common MPNs, and MDS/MPNs. Treatment of MPNs is complicated by phenotypic mimicry, determinants of thrombotic risk, and molecular markers; however, risk prognostication tools are available. Dr. Altman elaborated on current and new drugs that target various mutations including Jak2 and telomerase inhibitors for myelofibrosis in older patients. The next case study presented by Dr. Alvarnas illustrated the diagnosis and management of HLH. Allogeneic hematopoietic cell transplant (HCT) is the treatment for primary HLH whereas treatment for secondary HLH is based upon the underlying disorder.

The case studies segued to three talks encompassing AML, MDS, and ALL and the importance of cytogenetics and molecular genetics. Dr. Altman highlighted FLT3-ITD, NPM1, CEBPα, and C-KIT as important mutations in AML; future mutations of importance may include IDH1/2, DNMT2A, TET2 and ASXL1. Dr. Greenberg also identified molecular mutations in patients with MDS; however, these mutations are also seen in older patients without hematologic abnormalities and therefore must be interpreted with caution. Currently, mutations can guide treatment decisions but are not diagnostic, but mutations resulting in defective DNA damage repair could be targeted in the future, said Dr. Greenberg. Other factors important in treatment include stratification and delineation of treatment-related MDS (t-MDS) and de novo MDS. Patients with t-MDS have a poorer outcome and are less responsive to treatments.

Dr. Alvarnas discussed improved outcomes for ALL including optimization and application of current modalities and the incorporation of newer treatment options. Dr. Alvarnas discussed the broader application of pediatric regiments to other age groups and noted that allogeneic HCT should be considered in older patients who meet the fitness requirements. Finally, the clinical trial data that led to the FDA approval of blinatumomab for relapsed disease was presented and inotuzumab ozogamicin and CAR T-cells were discussed as developing therapies. 

With the introduction of new therapeutic options that could result in more curative therapy, late effects remain an important consideration in the growing population of survivors, creating a good segue into the next discussion in which Glen J. Peterson, RN, DNP, ACNP, University of Colorado Cancer Center, addressed the importance of developing a survivorship program for the management of long-term and late effects of curative therapy in lymphoma survivors.

The afternoon session commenced with patient case-based discussions on rare plasma cell disorders by panelists Dr. Damian Green, MD, Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance, and Dr. Amrita Krishnan, MD, City of Hope Comprehensive Cancer Center. The panelists provided rationale and clinical insights for diagnosis and treatment of amyloidosis, POEMS syndrome, and plasmacytoma.

Next, Steven P. Treon, MD, PhD, Dana-Farber/Brigham and Women's Cancer Center | Massachusetts General Hospital Cancer Center introduced the audience to new findings in the management of WM/LPL. He noted that MYD88 (L265P) mutations are present in greater than 95-97% of patients with WM and allele-specific polymerase chain reaction for MYD88 (L265P) has been adopted as an essential test in differentiating WM from other entities that secrete IgM. He also discussed clinical data on currently available combination therapies and on the newer therapeutic options for WM/LPL. In closing, Dr. Treon shared how genomics (mutations in MYD88 and CXCR4) helps identify patients who may benefit with ibrutinib treatment using a recently published study (Treon et al, N Engl J Med. 2015; 372(15):1430-40) as an example.

Dr. Green provided an overview of the management of MM, pointing out that the rapid changes in the treatment landscape have contributed to dramatic improvement in survival rates of patients with MM. He discussed the updated diagnostic criteria by the IMWG which includes: imaging, bone marrow assessment, and free light chain ratio existing requirements of CRAB features and how it redefines the subset of patients with smoldering myeloma requiring active treatment. Dr. Green also noted that patients with newly diagnosed disease are best served with a triplet regimen and treatment based on cytogenetic risk assessment may improve outcomes for individuals with high risk features. Autologous HCT remains the standard of care in transplant eligible patients. Dr. Green concluded his talk noting that new targets, immunotherapies and other novel approaches may be approved for MM treatment in the near future.

Bone-related morbidity is a debilitating side effect of MM, and Dr. Krishnan discussed the strategies for the management of bone health in patients, including surgical options and bisphosphonates. She provided an overview of clinical trial data showing the equivalence of pamidronate versus zoledronic acid, discussed data showing the survival advantage with zoledronic acid compared with clodronate, and talked about the potential role of denosumab in this setting. In conclusion, Dr. Krishnan presented novel approaches that are under clinical investigation for management of MM bone disease.

Dr. Abramson discussed the classification, clinical presentation and management of Castleman’s disease, a rare heterogeneous non-malignant lymphoproliferative disease. Unicentric Castleman’s disease is managed with surgical excision.  Systemic therapy is reserved for unresectable and relapsed/refractory disease. Multicentric Castleman’s disease (MCD) is a relapsing remitting disease and treatment is indicated when active disease is present. Siltuximab, an anti-IL-6 antibody, has a role in the treatment of MCD in HHV8/HIV negative patients, Dr. Abramson concluded. 

The final presentations focused on CHL. Dr. Advani reviewed risk-adapted strategies for optimizing front-line therapy for advanced stage CHL, emphasizing the need to use the Deauville criteria (5-point scale based on the assessment of uptake in the liver and mediastinum) for the interpretation of PET/CT.  A dialog between the medical oncologist and nuclear medicine physician is critical to appropriately implement the new response criteria to optimize treatment of advanced stage CHL, she explained. Weiyun Z. Ai, MD, PhD, UCSF Helen Diller Family Comprehensive Cancer Center, discussed the results of the pivotal clinical studies that formed the basis for the approval of brentuximab vedotin for relapsed/refractory CHL and as maintenance therapy after autologous HCT in patients at high risk of relapse.  Dr. Ai concluded her presentation mentioning that PD-1 blockade with nivolumab and pembrolizumab looks promising as a potential treatment option for heavily pretreated relapsed/ refractory HL.

The congress concluded with closing remarks from Dr. Zelenetz and Dr. Advani.

For more information about the NCCN Annual Congress: Hematologic Malignancies, visit NCCN.org/HEM.

Look for further announcements in upcoming editions of NCCN eBulletin regarding continuing education opportunities based on presentations from the NCCN 10th Annual Congress: Hematologic Malignancies™.