A comprehensive overview pdf author andreas engert isbn 3319125044 file size 8. In the united states in 20, about 9,290 persons were expected to be diagnosed with hodgkin lymphoma. Etiology of hodgkin lymphoma c81 in central and south. Classic hodgkin lymphoma chl is a monoclonal lymphoid neoplasm in most instances derived from b cells composed of mononuclear hodgkin and multinucleated hodgkin reedsternberg hrs cells residing in an infiltrate containing a variable mixture of nonneoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen fibers who classification. The understanding of the molecular pathogenesis of nonhodgkins lymphomas nhl has significantly improved in recent years.
Lymphomas are types of cancer that develop from lymphocytes, a type. The lymphatic system is part of your immune system. Novel developments in the pathogenesis and diagnosis of extranodal marginal zone lymphoma max i. Lymphocytes work together with other cells in the immune system to defend the body against invasion by bacteria, viruses, parasites, and other foreign substances. Only within the past 10 years has the bcell nature of the. Clear fluid called lymph flows through the lymphatic vessels and contains infectionfighting white blood cells, known as lymphocytes.
Il21 is expressed in hodgkin lymphoma and activates stat5. Advances in molecular biology and genetics lead to the identification and characterization of several oncogenic pathways involved in lymphomagenesis. Contribution of epsteinbarr virus latent proteins to the. Pathogenesis, diagnosis, and treatment of composite.
According to the who classification, hodgkins lymphoma hl is subdivided into a classical variant and a nodular lymphocyte predominant. Lymphoma is a cancer of a part of the immune system called the lymph system. This knowledge will ultimately lead to improved diagnostic and therapeutic strategies for patients with. Pathophysiology and molecular aspects of diffuse large b. Symptoms include painless lymphadenopathy, sometimes with fever, night sweats, unintentional weight loss, pruritus. The disease is defined in terms of its microscopic appearance histology see the image below and the expression of cell surface markers immunophenotype. In brazil, it is the fifth cause of cancer, with more than 55,000 cases and 26,000 deaths per year. A20 and rbx1 regulate brentuximab vedotin sensitivity in. Hodgkins lymphoma is a rare cancer of the lymphatic system that affects the blymphocytes and leaves a patient susceptible to infection. Patients are commonly diagnosed with hl in their 20s and 30s, and they present with supradiaphragmatic lymphadenopathy, often with systemic b.
Lymphoma is a general name for a group of cancers that affect the lymphatic system. Classical hodgkin lymphoma hl is characterized by the presence of reedsternberg rs cells, which are transformed. Two of the rare subtypes, marginal zone lymphoma mzl and mantle cell lymphoma mcl, make up approximately 8% and 4% of. Potential role of hypoxia in early stages of hodgkin lymphoma pathogenesis. Hodgkin lymphoma is a rather rare malignancy in the pediatric population, however, it constitutes approximately 40% of all lymphomas that present during childhood and is the most common malignancy in adolescents and young adults. A typical question patients with nonhodgkin lymphoma ask their physicians is what caused this cancer. Hodgkin lymphoma accounts for up to 1520% of all lymphomas in some western countries, but accounts for less than 10% of all lymphomas in asia, such as taiwan, japan and china. Approximately 8% of all nonhodgkin lymphomas are extranodal marginal zone b cell lymphoma of mucosa associated lymphoid tissue malt, also known as malt lymphoma, which was first described in 1983 by isaacson and wright.
Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. Pathogenesis of classical and lymphocytepredominant. Such composite lymphomas can be combinations of two nonhodgkin lymphomas or a combination of a nonhodgkin lymphoma and a hodgkins. Hodgkin lymphoma is an uncommon cancer that develops in the lymphatic system, which is a network of vessels and glands spread throughout your body. In more advanced cases of hodgkins lymphoma, several lymph nodes from the same group may become matted together, as seen in this group of mediastinal lymph nodes. Nonhodgkin lymphoma nhl is a group of blood cancers that includes all types of lymphoma except hodgkin lymphomas. Hodgkin lymphoma is a localized or disseminated malignant proliferation of cells of the lymphoreticular system, primarily involving lymph node tissue, spleen, liver, and bone marrow. What is the pathogenesis of classical hodgkin lymphoma. Natural history hodgkin lymphoma arises in a single node or chain of nodes and spreads first to anatomically contiguous lymphoid tissue. Nonhodgkin lymphomas hematology and oncology merck. Molecular pathogenesis of hodgkin lymphoma springerlink. Classical hodgkin lymphoma hl is characterized by the presence of reed sternberg rs cells, which are transformed postgerminal center.
Diffuse large bcell lymphoma is the most common subtype of nonhodgkin lymphoma in the west. Hodgkin lymphoma formerly, hodgkin disease is a potentially curable lymphoma with distinct histology, biologic behavior, and clinical characteristics. In hl, the tumor cells hodgkin and reedsternberg hrs cells are usually very rare in the tissue. Often there will be nonpainful enlarged lymph nodes in the neck, under the arm, or in the groin.
Novel developments in the pathogenesis and diagnosis of. Immunobiology and pathophysiology of hodgkin lymphomas. Hl is characterized by the giant multinucleated tumor cells called hodgkinreedsternberg hrs cells, but the cellular origin had long been unknown. Pathophysiology of hodgkin lymphoma mdedge hematology. Other symptoms may include bone pain, chest pain or itchiness. The most common subtype, diffuse large bcell lymphoma dlbcl, is characterized by a diffuse proliferation of large and mature bcells. Pdf classical hodgkin lymphoma hl is characterized by the presence of reedsternberg rs cells, which are transformed postgerminal center b cells. Such composite lymphomas can be combinations of two nonhodgkin lymphomas or a combination of a nonhodgkin lymphoma and a hodgkins lymphoma. Molecular pathogenesis of hodgkins lymphoma pg cme. Symptoms may include fever, night sweats, and weight loss. This book examines in detail the current treatment options for firstline, relapsed, and refractory hodgkin lymphoma and the management appropriate in.
Estimates suggest that around 1 in 25,000 people are. Hodgkin lymphoma hematology and oncology merck manuals. Case challenges in optimizing therapy for patients with. Nonhodgkin lymphomas nhls are a highly heterogeneous group of lymphoproliferative disorders that include both aggressive and indolent subtypes. Hodgkin lymphoma hodgkin lymphoma hl, one of the most curable forms of cancer, was named for thomas hodgkin, a british pathologist. Visceral involvement by hodgkin lymphoma may be secondary to extension from adjacent lymph nodes. Pier francesco ferrucci1 and emanuele zucca2 1department of haematoncology, european institute of oncology ieo, milan, italy, and 2department of oncology, southern switzerland institute of oncology iosi, s. Hodgkin lymphoma is a cancer of the immune system that is marked by the presence of a type of cell called the reedsternberg cell. The bcell nonhodgkin lymphomas bnhl are a diverse group of haematological malignancies which arise from the mature blymphocyte compartment.
The two major types of hodgkin lymphoma are classical hodgkin lymphoma and nodular lymphocytepredominant hodgkin lymphoma. The present chapter will outline key principles and general mechanisms underlying the pathogenesis of bcell nonhodgkin lymphomas in children and adolescents. The aetiology of this lymphoma, however, remained enigmatic for a long time. Section of cancer surveillance, international agency for. The molecular pathogenesis of hodgkin lymphoma wiley online. Nodular sclerosis2 classical hodgkins lymphoma, nodular sclerosis. Does q fever contribute to pathogenesis of nonhodgkin. Pathogenesis, diagnosis, and treatment of composite lymphomas. Pdf immunobiology and pathophysiology of hodgkin lymphomas. A20 and rbx1 regulate brentuximab vedotin sensitivity in hodgkin lymphoma models wei wei 1, yuquan lin 2, zhihui song 1, wenming xiao 3, liqi chen 1, jiejing yin 1, yan zhou 4, stefan k. In hodgkins lymphoma hl, the b cell origin of the tumour cells, the hodgkin and reedsternberg hrs cells, has been disclosed by molecular single cell analysis about 10 yr ago. Pathogenesis of hodgkins lymphoma request pdf researchgate.
Recently, our understanding of the molecular pathogenesis of these disorders has greatly increased due to technological advances such as highthroughput dna sequencing techniques. Hodgkin lymphoma hl is composed of two distinct pathological entities, nodular lymphocyte predominant hl and classic hl, the latter with four subtyp. Epsteinbarr virusassociated hodgkins lymphoma survivors of hodgkins lymphoma hl frequently have many years to experience the longterm toxicities of combined modality therapies. Both subtypes demonstrate a paucity of surreptitious in chl neoplastic b cells within a background of reactive inflammatory cells underscoring both the relatedness of these 2 entities to each other, as well as their.
This disease was called hodgkin s disease for about 170 years. Some bacterial and viral infectionseg, helicobacter pylori, chlamydia psittaci, epsteinbarr virus, and human lymphotropic virus type iare associated with development of some lymphomas. Hodgkin lymphoma hl is a type of lymphoma in which cancer originates from a specific type of white blood cells called lymphocytes. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss and tiredness. Hodgkin described several cases of people with symptoms of a cancer involving the lymph nodes. This lymphoid malignancy involves peripheral lymph nodes and can also affect organs such as liver, lung, and bone marrow.
About 40% of patients suffer from constitutional symptoms bsymptoms. Hodgkin lymphoma hl encompasses 2 unique clinicopathologic entities, classical hodgkin lymphoma chl. Presenting symptoms usually include peripheral lymphadenopathy. Hodgkins lymphoma is a rare cancer of the lymphatic system that affects groups of lymph nodes, such as those found in the neck, armpits or groin. The molecular pathogenesis of hodgkin lymphoma request pdf. Nonhodgkin lymphomas begin when a type of white blood cell, called a t cell or b cell, becomes abnormal. Hodgkin lymphoma hl is one of the most frequent lymphomas in the western world, with an annual incidence of about 3 cases per 100,000 persons. Malt lymphomas arise at a wide range of different extranodal sites, with the highest frequency in the stomach, followed by lung, ocular adnexa, and. Hodgkin lymphoma hl is a type of cancer that afects specialized white blood cells called lymphocytes. Composite lymphomas pose a particular diagnostic challenge, and there are currently no agreed standards for treatment.
In all age groups, hodgkin lymphoma is highly sensitive to chemotherapy and irradiation. Mass cytometry study of follicular lymphoma tumors reveals cellular characteristics. In classical hodgkin lymphoma, the neoplastic cell is the reedsternberg cell see the image below. Nonhodgkin lymphomas nhls occur as a result of an expansion and progressive accumulation of a mature single clone of lymphocytes. Hodgkin lymphoma hl is a distinctive lymphoma subtype that accounts for approximately 30% of all lymphomas in the western world and approximately 5% in japan. In rare instances, two distinct lymphomas concurrently occur in a patient. Some forms are slowgrowing, while others are fastgrowing. Hodgkin lymphoma hl is a unique hematopoietic neoplasm characterized by cancerous reed. A few cases of combined classic and nodular lymphocyte predominant hodgkins lymphoma nlphl, the two major subtypes of hodgkins lymphoma, have also been described. The escape from apoptosis and transcriptional reprogramming of hrs cells are interlinked and appear central to disease pathogenesis. It represents the third most common non hodgkin lymphoma in the western world, and the median age of occurrence is around 60 years. After the identification of the ma lignant, clonal expansion of b cells in the pathogenesis, the dis ease was named hodgkin lymphoma hl 4. Request pdf pathogenesis of hodgkins lymphoma in hodgkins lymphoma hl, the b cell origin of the tumour cells, the hodgkin and. Nonhodgkin lymphoma nhl consists of a diverse group of malignant tumors of the lymphoid tissues variously derived from the clonal expansion of b cells, t cells, natural killer nk cells or precursors of these cells.
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