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British Journal of Haematology Jan 2019The Hodgkin lymphomas are a family of unique lymphoma subtypes, in which the nature of the neoplastic cell was enigmatic for many years. Much of the mystery has been... (Review)
Review
The Hodgkin lymphomas are a family of unique lymphoma subtypes, in which the nature of the neoplastic cell was enigmatic for many years. Much of the mystery has been solved, with all forms now considered to be of B-cell origin, in most cases of germinal centre derivation. Today we recognize Hodgkin lymphoma as an eponym that encompasses multiple entities. One of the unifying themes is the major contribution from the tumour microenvironment. Both the character of the neoplastic cells and the nature of the immune environment are critical to accurate diagnosis. Moreover, an understanding of the molecular alterations that characterize both the neoplastic cells and their microenvironment have led to therapeutic advances, targeting both neoplastic and reactive components. Other conditions may foster a similar inflammatory milieu and lead to lymphoproliferations that mimic the Hodgkin lymphomas. In this review we provide an update on the diagnostic features of the various subtypes and include additional information relevant for prognostic evaluation and investigation of potential therapeutic targets. Additionally, we also discuss those conditions that often cause confusion in diagnosis and need to be distinguished from the Hodgkin lymphomas.
Topics: Diagnosis, Differential; Hodgkin Disease; Humans; Tumor Microenvironment
PubMed: 30407610
DOI: 10.1111/bjh.15614 -
Blood Dec 2020Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity with distinct pathologic and clinical characteristics. Unlike the malignant cells in... (Review)
Review
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity with distinct pathologic and clinical characteristics. Unlike the malignant cells in classical Hodgkin lymphoma, the disease-defining lymphocyte-predominant cells in NLPHL are consistently positive for CD20, but do not express CD30. The clinical course of NLPHL is indolent in the majority of cases. Most patients present with early-stage disease at the initial diagnosis. First-line treatment of stage IA NLPHL usually consists of limited-field radiotherapy alone. Patients with early-stage NLPHL other than stage IA and intermediate-stage disease mostly receive combined-modality treatment, whereas individuals with advanced NLPHL are treated with chemotherapy alone. In relapsed NLPHL, conventional chemotherapy, anti-CD20 antibodies, and radiotherapy represent active treatment modalities. Only patients with poor-risk characteristics such as early disease recurrence are candidates for aggressive salvage treatment with high-dose chemotherapy and autologous stem cell transplantation. The overall and relative survival of patients with NLPHL is excellent as indicated by a low excess mortality compared with the general population. This article discusses treatment options for patients with NLPHL and factors that influence the choice of therapy on the basis of the available data and 2 clinical cases.
Topics: Adult; Combined Modality Therapy; Hodgkin Disease; Humans; Male; Recurrence
PubMed: 32877522
DOI: 10.1182/blood.2019004044 -
American Journal of Hematology Nov 2022Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8540 new patients annually and representing approximately 10% of all lymphomas in the United...
DISEASE OVERVIEW
Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8540 new patients annually and representing approximately 10% of all lymphomas in the United States.
DIAGNOSIS
HL is composed of two distinct disease entities: classical HL and nodular lymphocyte-predominant HL. Nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich HL are subgroups of classical HL.
RISK STRATIFICATION
An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy. Prognostic models that identify patients at low or high risk for recurrence, as well as the response to therapy as determined by positron emission tomography scan, are used to optimize therapy.
RISK-ADAPTED THERAPY
Initial therapy for HL patients is based on the histology of the disease, the anatomical stage, and the presence of poor prognostic features. Patients with early-stage disease are typically treated with combined modality strategies utilizing abbreviated courses of combination chemotherapy followed by involved-field radiation therapy, while those with advanced-stage disease receive a longer course of chemotherapy, often without radiation therapy. However, newer agents, including brentuximab vedotin and anti-programmed death-1 (PD-1) antibodies, are now being incorporated into frontline therapy.
MANAGEMENT OF RELAPSED/REFRACTORY DISEASE
High-dose chemotherapy (HDCT) followed by an autologous stem cell transplant (ASCT) is the standard of care for most patients who relapse following initial therapy. For patients who fail HDCT with ASCT, brentuximab vedotin, PD-1 blockade, non-myeloablative allogeneic transplant, or participation in a clinical trial should be considered.
Topics: Antineoplastic Combined Chemotherapy Protocols; Brentuximab Vedotin; Disease Management; Hodgkin Disease; Humans; Immunoconjugates; Neoplasm Recurrence, Local; Programmed Cell Death 1 Receptor
PubMed: 36215668
DOI: 10.1002/ajh.26717 -
CA: a Cancer Journal For Clinicians Mar 2018Hodgkin lymphoma (HL) is a unique hematopoietic neoplasm characterized by cancerous Reed-Sternberg cells in an inflammatory background. Patients are commonly diagnosed... (Review)
Review
Hodgkin lymphoma (HL) is a unique hematopoietic neoplasm characterized by cancerous Reed-Sternberg cells in an inflammatory background. Patients are commonly diagnosed with HL in their 20s and 30s, and they present with supradiaphragmatic lymphadenopathy, often with systemic B symptoms. Even in advanced-stage disease, HL is highly curable with combination chemotherapy, radiation, or combined-modality treatment. Although the same doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapeutic regimen has been the mainstay of therapy over the last 30 years, risk-adapted approaches have helped de-escalate therapy in low-risk patients while intensifying treatment for higher risk patients. Even patients who are not cured with initial therapy can often be salvaged with alternate chemotherapy combinations, the novel antibody-drug conjugate brentuximab, or high-dose autologous or allogeneic hematopoietic stem cell transplantation. The programmed death-1 inhibitors nivolumab and pembrolizumab have both demonstrated high response rates and durable remissions in patients with relapsed/refractory HL. Alternate donor sources and reduced-intensity conditioning have made allogeneic hematopoietic stem cell transplantation a viable option for more patients. Future research will look to integrate novel strategies into earlier lines of therapy to improve the HL cure rate and minimize long-term treatment toxicities. CA Cancer J Clin 2018;68:116-132. © 2017 American Cancer Society.
Topics: Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Combined Modality Therapy; Diagnosis, Differential; Diagnostic Imaging; Hematopoietic Stem Cell Transplantation; Hodgkin Disease; Humans; Neoplasm Staging; Prognosis; Risk Factors; Survival Analysis; Transplantation Conditioning
PubMed: 29194581
DOI: 10.3322/caac.21438 -
American Journal of Hematology Aug 2020Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8480 new patients annually and representing approximately 10% of all lymphomas in the United...
DISEASE OVERVIEW
Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8480 new patients annually and representing approximately 10% of all lymphomas in the United States.
DIAGNOSIS
Hodgkin lymphoma is composed of two distinct disease entities: classical HL and nodular lymphocyte predominant HL. Nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich HL are subgroups of classical HL.
RISK STRATIFICATION
An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy. Prognostic models that identify patients at low or high risk for recurrence, as well as the response to therapy as determined by positron emission tomography (PET) scan, are used to optimize therapy.
RISK-ADAPTED THERAPY
Initial therapy for HL patients is based on the histology of the disease, the anatomical stage and the presence of poor prognostic features. Patients with early stage disease are typically treated with combined modality strategies utilizing abbreviated courses of combination chemotherapy, followed by involved-field radiation therapy. Patients with advanced stage disease receive a longer course of chemotherapy, often without radiation therapy. However, newer agents including brentuximab vedotin and anti-PD-1 antibodies are now being incorporated into frontline therapy.
MANAGEMENT OF RELAPSED/REFRACTORY DISEASE
High-dose chemotherapy (HDCT) followed by an autologous stem cell transplant (ASCT) is the standard of care for most patients who relapse following initial therapy. For patients who fail HDCT with ASCT, brentuximab vedotin, PD-1 blockade, non-myeloablative allogeneic transplant or participation in a clinical trial should be considered.
Topics: Female; History, 21st Century; Hodgkin Disease; Humans; Male; Middle Aged; Prognosis
PubMed: 32384177
DOI: 10.1002/ajh.25856 -
Blood Aug 2019Although a pathogenic role for the Epstein-Barr virus (EBV) is largely undisputed for tumors that are consistently EBV genome positive (eg, nasopharyngeal carcinoma,... (Review)
Review
Although a pathogenic role for the Epstein-Barr virus (EBV) is largely undisputed for tumors that are consistently EBV genome positive (eg, nasopharyngeal carcinoma, endemic Burkitt lymphoma), this is not the case for classical Hodgkin lymphoma (cHL), a tumor with only a variable EBV association. In light of recent developments in immunotherapeutics and small molecules targeting EBV, we believe it is now timely to reevaluate the role of EBV in cHL pathogenesis.
Topics: Animals; Epstein-Barr Virus Infections; Gene Expression Regulation, Viral; Genes, Viral; Herpesvirus 4, Human; Hodgkin Disease; Humans; Risk Factors
PubMed: 31186275
DOI: 10.1182/blood.2019000568 -
CA: a Cancer Journal For Clinicians 1993The etiology of Hodgkin's disease is unknown, and the identity of the normal counterpart of the malignant cell involved in Hodgkin's disease remains controversial. Yet,... (Review)
Review
The etiology of Hodgkin's disease is unknown, and the identity of the normal counterpart of the malignant cell involved in Hodgkin's disease remains controversial. Yet, three of every four patients diagnosed with Hodgkin's disease will be cured. This success in treatment is due in large part to the application of modern oncologic techniques, such as combination chemotherapy and the use of staging to guide therapy. This article reviews important advances in the understanding and treatment of Hodgkin's disease.
Topics: Acquired Immunodeficiency Syndrome; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Female; Hodgkin Disease; Humans; Neoplasm Staging; Pregnancy; Pregnancy Complications, Neoplastic; Prognosis; Salvage Therapy
PubMed: 8242436
DOI: 10.3322/canjclin.43.6.327 -
Lancet (London, England) Sep 2012Management of Hodgkin's lymphoma continues to develop. Outcomes for patients with favourable-risk, early-stage disease are excellent, and serial reductions in intensity... (Review)
Review
Management of Hodgkin's lymphoma continues to develop. Outcomes for patients with favourable-risk, early-stage disease are excellent, and serial reductions in intensity of treatment have been made to retain the excellent prognosis while reducing the late effects of treatment. Prognosis is also very good in advanced-stage disease but the rate of relapse is higher than in early-stage disease, and the optimum first-line treatment is unclear. Workers are investigating the role of functional imaging to assess whether treatment can be tailored according to response, with the most intensive therapies reserved for patients predicted to have poor outcomes. In this Seminar we critically appraise the management of Hodgkin's lymphoma in early-stage disease, advanced-stage disease, and at relapse, with a focus on late effects of treatment.
Topics: Adult; Combined Modality Therapy; Hodgkin Disease; Humans; Neoplasm Staging; Positron-Emission Tomography; Prognosis; Recurrence; Salvage Therapy; Treatment Outcome
PubMed: 22835602
DOI: 10.1016/S0140-6736(12)60035-X -
British Journal of Haematology Jan 2019Hodgkin lymphoma (HL) commonly occurs in adolescents and young adults (AYA), defined by the National Cancer Institute as people diagnosed with cancer between the ages of... (Comparative Study)
Comparative Study Review
Hodgkin lymphoma (HL) commonly occurs in adolescents and young adults (AYA), defined by the National Cancer Institute as people diagnosed with cancer between the ages of 15 and 39 years. Despite therapeutic advances, the AYA population has derived less incremental benefit compared to both paediatric and adult counterparts. Although the exact aetiology is unclear, contributing factors probably include differences in disease biology, delayed diagnosis, decreased participation in clinical trials and treatment adherence secondary to complex social factors. As such, while HL remains highly curable, there is not a clear consensus regarding the management of patients within this age range, specifically whether paediatric or adult regimens are preferred or how best to incorporate emerging therapeutic advancements. Ongoing clinical trials, as well as continued collaborative efforts are required to address the needs of this population, investigate the potential for unique biological factors and allow for optimization of treatment. Here we review current prognostic and treatment strategies for paediatric and adult patients with HL and highlight complexities around the management of this patient population.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Female; Hodgkin Disease; Humans; Male; Medication Adherence; Socioeconomic Factors; Young Adult
PubMed: 30460695
DOI: 10.1111/bjh.15640 -
Clinical Medicine (London, England) May 2022Accelerated coronary artery disease seen following radiation exposure is termed 'radiation-induced coronary artery disease' (RICAD) and results from both the direct and... (Review)
Review
Accelerated coronary artery disease seen following radiation exposure is termed 'radiation-induced coronary artery disease' (RICAD) and results from both the direct and indirect effects of radiation exposure. Long-term data are available from survivors of nuclear explosions and accidents, nuclear workers as well as from radiotherapy patients. The last group is, by far, the biggest cause of RICAD presentation.The incidence of RICAD continues to increase as cancer survival rates improve and it is now the second most common cause of morbidity and mortality in patients treated with radiotherapy for breast cancer, Hodgkin's lymphoma and other mediastinal malignancies. RICAD will frequently present atypically or even asymptomatically with a latency period of at least 10 years after radiotherapy treatment. An awareness of RICAD, as a long-term complication of radiotherapy, is therefore essential for the cardiologist, oncologist and general medical physician alike.Prior cardiac risk factors, a higher radiation dose and a younger age at exposure seem to increase a patient's risk ratio of developing RICAD. Significant radiation exposure, therefore, requires a low threshold for screening for early diagnosis and timely intervention.
Topics: Coronary Artery Disease; Hodgkin Disease; Humans; Incidence; Risk Factors; Survival Rate
PubMed: 35584837
DOI: 10.7861/clinmed.2021-0600