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. Pathogenesis, diagnosis, and treatment of composite lymphomas. Nonhodgkin lymphomas nhls occur as a result of an expansion and progressive accumulation of a mature single clone of lymphocytes. Symptoms include painless lymphadenopathy, sometimes with fever, night sweats, unintentional weight loss, pruritus. The two major types of lymphoma are hodgkin lymphoma hl and nonhodgkin lymphoma nhl.
Novel developments in the pathogenesis and diagnosis of extranodal marginal zone lymphoma max i. A20 and rbx1 regulate brentuximab vedotin sensitivity in. Nonhodgkin lymphoma nhl is a group of blood cancers that includes all types of lymphoma except hodgkin lymphomas. 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. Pathogenesis of hodgkins lymphoma request pdf researchgate. In brazil, it is the fifth cause of cancer, with more than 55,000 cases and 26,000 deaths per year. After the identification of the ma lignant, clonal expansion of b cells in the pathogenesis, the dis ease was named hodgkin lymphoma hl 4. A recurrent 11q aberration pattern characterizes a subset of myc.
Hodgkin lymphoma hl is composed of two distinct pathological entities, nodular lymphocyte predominant hl and classic hl, the latter with four subtyp. 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. A typical question patients with nonhodgkin lymphoma ask their physicians is what caused this cancer. Immunobiology and pathophysiology of hodgkin lymphomas. In rare instances, two distinct lymphomas concurrently occur in a patient. Hodgkin lymphoma hl is a type of lymphoma in which cancer originates from a specific type of white blood cells called lymphocytes. The cell divides again and again, making more and more. Classical hodgkin lymphoma hl is characterized by the presence of reed sternberg rs cells, which are transformed postgerminal center. This lymphoid malignancy involves peripheral lymph nodes and can also affect organs such as liver, lung, and bone marrow. Diffuse large bcell lymphoma is the most common subtype of nonhodgkin lymphoma in the west. Primary gastric lymphoma pathogenesis and treatment. The escape from apoptosis and transcriptional reprogramming of hrs cells are interlinked and appear central to disease pathogenesis. Hl is characterized by the giant multinucleated tumor cells called hodgkinreedsternberg hrs cells, but the cellular origin had long been unknown. Hodgkin lymphoma hodgkin lymphoma hl, one of the most curable forms of cancer, was named for thomas hodgkin, a british pathologist.
The present chapter will outline key principles and general mechanisms underlying the pathogenesis of bcell nonhodgkin lymphomas in children and adolescents. Hodgkin lymphoma hl is a unique hematopoietic neoplasm characterized by cancerous reed. The disease is defined in terms of its microscopic appearance histology see the image below and the expression of cell surface markers immunophenotype. Hodgkin lymphoma formerly, hodgkin disease is a potentially curable lymphoma with distinct histology, biologic behavior, and clinical characteristics. 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.
Other symptoms may include bone pain, chest pain or itchiness. This disease was called hodgkin s disease for about 170 years. In classical hodgkin lymphoma, the neoplastic cell is the reedsternberg cell see the image below. Only within the past 10 years has the bcell nature of the. Nonhodgkin lymphomas begin when a type of white blood cell, called a t cell or b cell, becomes abnormal. Pathophysiology of hodgkin lymphoma mdedge hematology.
This knowledge will ultimately lead to improved diagnostic and therapeutic strategies for patients with. Estimates suggest that around 1 in 25,000 people are. Pdf immunobiology and pathophysiology of hodgkin lymphomas. Etiology of hodgkin lymphoma c81 in central and south.
Some bacterial and viral infectionseg, helicobacter pylori, chlamydia psittaci, epsteinbarr virus, and human lymphotropic virus type iare associated with development of some lymphomas. Il21 is expressed in hodgkin lymphoma and activates stat5. 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 understanding of the molecular pathogenesis of nonhodgkins lymphomas nhl has significantly improved in recent years. Composite lymphomas pose a particular diagnostic challenge, and there are currently no agreed standards for treatment. Hodgkins lymphoma is a rare cancer of the lymphatic system that affects the blymphocytes and leaves a patient susceptible to infection. Malt lymphomas arise at a wide range of different extranodal sites, with the highest frequency in the stomach, followed by lung, ocular adnexa, and. A few cases of combined classic and nodular lymphocyte predominant hodgkins lymphoma nlphl, the two major subtypes of hodgkins lymphoma, have also been described. Some forms are slowgrowing, while others are fastgrowing.
Two of the rare subtypes, marginal zone lymphoma mzl and mantle cell lymphoma mcl, make up approximately 8% and 4% of. Lymphomas are types of cancer that develop from lymphocytes, a type. Clear fluid called lymph flows through the lymphatic vessels and contains infectionfighting white blood cells, known as lymphocytes. Pathogenesis of classical and lymphocytepredominant. 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. Nonhodgkin lymphomas nhls are a highly heterogeneous group of lymphoproliferative disorders that include both aggressive and indolent subtypes.
Such composite lymphomas can be combinations of two nonhodgkin lymphomas or a combination of a nonhodgkin lymphoma and a hodgkins. According to the who classification, hodgkins lymphoma hl is subdivided into a classical variant and a nodular lymphocyte predominant. Does q fever contribute to pathogenesis of nonhodgkin. Contribution of epsteinbarr virus latent proteins to the. Symptoms may include fever, night sweats, and weight loss. 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. Such composite lymphomas can be combinations of two nonhodgkin lymphomas or a combination of a nonhodgkin lymphoma and a hodgkins lymphoma.
The molecular pathogenesis of hodgkin lymphoma wiley online. Lymphoma is a general name for a group of cancers that affect the lymphatic system. Hrs cells have, however, largely lost their b cell phenotype and aberrantly express markers and transcriptional regulators of other hematolymphoid cell types. Classical hodgkin lymphoma hl is characterized by the presence of reedsternberg rs cells, which are transformed. Nonhodgkin lymphomas hematology and oncology merck. Epsteinbarr virusassociated hodgkins lymphoma survivors of hodgkins lymphoma hl frequently have many years to experience the longterm toxicities of combined modality therapies. What is the pathogenesis of classical hodgkin lymphoma.
Lymphocytes work together with other cells in the immune system to defend the body against invasion by bacteria, viruses, parasites, and other foreign substances. Novel developments in the pathogenesis and diagnosis of. The bcell nonhodgkin lymphomas bnhl are a diverse group of haematological malignancies which arise from the mature blymphocyte compartment. Lymphoma is a cancer of a part of the immune system called the lymph system. Hodgkin lymphoma hl, a b cellderived cancer, is one of the most common lymphomas. The molecular pathogenesis of hodgkin lymphoma request pdf. Hodgkin lymphoma is a cancer of the immune system that is marked by the presence of a type of cell called the reedsternberg cell. Nodular sclerosis2 classical hodgkins lymphoma, nodular sclerosis. Hodgkin lymphoma hl is a type of cancer that afects specialized white blood cells called lymphocytes. Molecular pathogenesis of hodgkin lymphoma springerlink. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss and tiredness. A comprehensive overview pdf author andreas engert isbn 3319125044 file size 8. Case challenges in optimizing therapy for patients with. Pathogenesis, diagnosis, and treatment of composite.
Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. 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. Section of cancer surveillance, international agency for. 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. 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. Often there will be nonpainful enlarged lymph nodes in the neck, under the arm, or in the groin. 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. Patients are commonly diagnosed with hl in their 20s and 30s, and they present with supradiaphragmatic lymphadenopathy, often with systemic b. Nonhodgkin lymphomas nhl are a heterogeneous group of disorders involving malignant monoclonal proliferation of lymphoid cells in lymphoreticular sites, including lymph nodes, bone marrow, the spleen, the liver, and the gi tract. It represents the third most common non hodgkin lymphoma in the western world, and the median age of occurrence is around 60 years. This book examines in detail the current treatment options for firstline, relapsed, and refractory hodgkin lymphoma and the management appropriate in. Hodgkin described several cases of people with symptoms of a cancer involving the lymph nodes. Pathophysiology and molecular aspects of diffuse large b. 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.
Visceral involvement by hodgkin lymphoma may be secondary to extension from adjacent lymph nodes. The lymphatic system is part of your immune system. Mass cytometry study of follicular lymphoma tumors reveals cellular characteristics. Request pdf pathogenesis of hodgkins lymphoma in hodgkins lymphoma hl, the b cell origin of the tumour cells, the hodgkin and. 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.
The two major types of hodgkin lymphoma are classical hodgkin lymphoma and nodular lymphocytepredominant hodgkin lymphoma. Advances in molecular biology and genetics lead to the identification and characterization of several oncogenic pathways involved in lymphomagenesis. Hodgkin lymphoma is an uncommon cancer that develops in the lymphatic system, which is a network of vessels and glands spread throughout your body. Molecular pathogenesis of hodgkins lymphoma pg cme. Natural history hodgkin lymphoma arises in a single node or chain of nodes and spreads first to anatomically contiguous lymphoid tissue.
Recently, our understanding of the molecular pathogenesis of these disorders has greatly increased due to technological advances such as highthroughput dna sequencing techniques. In the united states in 20, about 9,290 persons were expected to be diagnosed with hodgkin lymphoma. The aetiology of this lymphoma, however, remained enigmatic for a long time. The most common subtype, diffuse large bcell lymphoma dlbcl, is characterized by a diffuse proliferation of large and mature bcells. 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.
About 40% of patients suffer from constitutional symptoms bsymptoms. Hodgkin lymphoma hematology and oncology merck manuals. Potential role of hypoxia in early stages of hodgkin lymphoma pathogenesis. In all age groups, hodgkin lymphoma is highly sensitive to chemotherapy and irradiation.
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