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Lymphoma From Wikipedia, the free encyclopedia This article is about lymphoma in humans. For the disease in dogs, cats, and ferrets, see lymphoma in animals. This article may require copy editing for grammar, style, cohesion, tone or spelling. You can assist by editing it. (October 2009) Please help improve this article by expanding it. Further information might be found on the talk page. (January 2010) This article may require cleanup to meet Wikipedia's quality standards. Please improve this article if you can. (January 2010) Lymphoma Classification and external resources Gastric MALT lymphoma ICD-10 C81.-C96. ICD-O: 9590-9999 MeSH D008223 Lymphoma is a cancer that begins in the lymphocytes of the immune system and presents as a solid tumor of lymphoid cells. It is treatable with chemotherapy, and in some cases radiotherapy and/or bone marrow transplantation, and can be curable, depending on the histology, type, and stage of the disease.[1] These malignant cells often originate in lymph nodes, presenting as an enlargement of the node (a tumor). Lymphomas are closely related to lymphoid leukemias, which also originate in lymphocytes but typically involve only circulating blood and the bone marrow (where blood cells are generated in a process termed haematopoesis) and do not usually form static tumors.[1] There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases called hematological neoplasms. Thomas Hodgkin published the first description of lymphoma in 1832, specifically of the form named after him, Hodgkin's lymphoma.[2] Since then, many other forms of lymphoma have been described, grouped under several proposed classifications. The 1982 Working formulation classification became very popular. It introduced the category non-Hodgkin lymphoma (NHL), divided into 16 different diseases. However, because these different lymphomas have little in common with each other, the NHL label is of limited usefulness for doctors or patients and is slowly being abandoned. The latest classification by the WHO (2001) lists 43 different forms of lymphoma divided in four broad groups. Some forms of lymphoma are categorized as indolent (e.g. small lymphocytic lymphoma), compatible with a long life even without treatment, whereas other forms are aggressive (e.g. Burkitt's lymphoma), causing rapid deterioration and death. However, most of the aggressive lymphomas respond well to treatment and are curable. The prognosis therefore depends on the correct classification of the disease, established by a pathologist after examination of a biopsy.[3] Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. True histiocytic malignancies are rare and are classified as sarcomas.[4] Contents [hide] 1 Classification 1.1 REAL 1.2 WHO 1.2.1 Mature B cell neoplasms 1.2.2 Mature T cell and natural killer (NK) cell neoplasms 1.2.3 Hodgkin lymphoma 1.2.4 Immunodeficiency-associated lymphoproliferative disorders 1.3 Working Formulation and Non-Hodgkin lymphoma 1.4 Other classification systems 2 Symptoms 3 Diagnosis, etiology, staging, prognosis, and treatment 4 Epidemiology 5 See also 6 References 7 External links [edit]Classification A number of different classification systems exist for lymphoma. As an alternative to the American Lukes-Butler classification, in the early 1970s, Karl Lennert of Kiel, Germany, proposed a new system of classifying lymphomas based on cellular morphology and their relationship to cells of the normal peripheral lymphoid system.[5] [edit]REAL In the mid 1990s, the Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic entities among all the lymphomas except Hodgkin's lymphoma.[6] REAL has been superseded by the WHO classification. [edit]WHO The WHO Classification, published in 2001 and updated in 2008,[4] is the latest classification of lymphoma and is based upon the foundations laid within the "Revised European-American Lymphoma classification" (REAL). This system attempts to group lymphomas by cell type (i.e. the normal cell type that most resembles the tumor) and defining phenotypic, molecular or cytogenetic characteristics. There are three large groups: the B cell, T cell, and natural killer cell tumors. Other less common groups, are also recognized. Hodgkin's lymphoma, although considered separately within the WHO (and preceding) classifications, is now recognized as being a tumor of, albeit markedly abnormal, lymphocytes of mature B cell lineage. [edit]Mature B cell neoplasms DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. Colors indicate levels of expression; green indicates genes that are underexpressed in lymphoma cells (as compared to normal cells), whereas red indicates genes that are overexpressed in lymphoma cells. Chronic lymphocytic leukemia/Small lymphocytic lymphoma B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma (such as Waldenström macroglobulinemia) Splenic marginal zone lymphoma Plasma cell neoplasms: Plasma cell myeloma Plasmacytoma Monoclonal immunoglobulin deposition diseases Heavy chain diseases Extranodal marginal zone B cell lymphoma, also called MALT lymphoma Nodal marginal zone B cell lymphoma (NMZL) Follicular lymphoma Mantle cell lymphoma Diffuse large B cell lymphoma Mediastinal (thymic) large B cell lymphoma Intravascular large B cell lymphoma Primary effusion lymphoma Burkitt lymphoma/leukemia [edit]Mature T cell and natural killer (NK) cell neoplasms T cell prolymphocytic leukemia T cell large granular lymphocytic leukemia Aggressive NK cell leukemia Adult T cell leukemia/lymphoma Extranodal NK/T cell lymphoma, nasal type Enteropathy-type T cell lymphoma Hepatosplenic T cell lymphoma Blastic NK cell lymphoma Mycosis fungoides / Sezary syndrome Primary cutaneous CD30-positive T cell lymphoproliferative disorders Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis Angioimmunoblastic T cell lymphoma Peripheral T cell lymphoma, unspecified Anaplastic large cell lymphoma [edit]Hodgkin lymphoma Classical Hodgkin lymphomas: Nodular sclerosis Mixed cellularity Lymphocyte-rich Lymphocyte depleted or not depleted Nodular lymphocyte-predominant Hodgkin lymphoma [edit]Immunodeficiency-associated lymphoproliferative disorders Associated with a primary immune disorder Associated with the Human Immunodeficiency Virus (HIV) Post-transplant Associated with methotrexate therapy Primary central nervous system lymphoma occurs most often in immunocomprimised patients,in particular those with AIDS,but it can occur in the immunocompetent as well.It has a poor prognosis,particulary in those with AIDS.Treatment can consist of corticosteroids,radiotherapy,and chemotherapy,often with methotrexate. [edit]Working Formulation and Non-Hodgkin lymphoma The 1982 Working Formulation is a classification of non-Hodgkin lymphoma. It excluded the Hodgkin's lymphomas and divided the remaining lymphomas (currently numbering 39 distinct diseases) into four grades (Low, Intermediate, High, and Miscellaneous) related to prognosis, with some further subdivisions based on the size and shape of affected cells. Because the most sophisticated technology used in diagnostic work at the time was the simple light microscope, it included no information about cell surface markers, or genetics, and it made no distinction between T-cell lymphomas or B-cell lymphomas. The Working Forumulation was superseded by REAL, which in turn has since been replaced by the WHO in 2001 (updated in September 2008), but it is still used by cancer agencies for compilation of lymphoma statistics and historical comparisons. The Working Formulation is obsolete,[7] as is the term non-Hodgkin lymphoma. [edit]Other classification systems ICD-O (codes 9590-9999, details at [1]) (archive link, was dead) ICD-10 (codes C81-C96, details at [2]) [edit]Symptoms Lymphadenopathy Fever of unknown origin Night sweats Pruritus Weight loss Anorexia Dyspnea Fatigue[8] [edit]Diagnosis, etiology, staging, prognosis, and treatment This article does not cite any references or sources. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. Depends on the specific form of lymphoma. For low-grade lymphoma, watchful waiting is often the initial course of action. If a low-grade lymphoma is becoming symptomatic, radiotherapy or chemotherapy are the treatments of choice, as while they do not cure the lymphoma, they can palliate symptoms, particulary painful lymphadenopathy. Patients with low-grade lymphoma can live near-normal lifespans, but the disease is incurable. Treatment of high-grade lymphoma can result in a cure in the majority of cases, however, the prognosis for patients with a poor response to therapy is worse. Treatment for high-grade lymphoma typically consists of aggressive chemotherapy, including the CHOP regimen. Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized. Advanced Hodgkins disease requires systemic chemotherapy, sometimes combined with radiotherapy. See the article on the corresponding form of lymphoma for further information.[citation needed] [edit]Epidemiology Age-standardized death from lymphomas and multiple myeloma per 100,000 inhabitants in 2004.[9] no data less than 1.8 1.8-3.6 3.6-5.4 5.4-7.2 7.2-9 9-10.8 10.8-12.6 12.6-14.4 14.4-16.2 16.2-18 18-19.8 more than 19.8 Lymphoma is the most common form of hematological malignancy, or "blood cancer", in the developed world. Taken together, lymphomas represent 5.3% of all cancers (excluding simple basal cell and squamous cell skin cancers) in the United States, and 55.6% of all blood cancers.[10] According to the U.S. National Institutes of Health, lymphomas account for about five percent of all cases of cancer in the United States, and Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States. Because the whole system is part of the body's immune system, patients with a weakened immune system, such as from HIV infection or from certain drugs or medication, also have a higher incidence of lymphoma. [edit]See also Hodgkin's lymphoma Non-Hodgkin's lymphoma Follicular lymphoma Burkitt's lymphoma Mantle cell lymphoma Gastric lymphoma Cutaneous T Cell lymphoma Mycosis fungoides Anaplastic large cell lymphoma MALT lymphoma Primary central nervous system lymphoma BCP-1 cells Ann Arbor staging International Prognostic Index Epstein barr virus Lymphadenopathy Chemotherapy regimens [edit]References ^ a b Parham, Peter (2005). The immune system. New York: Garland Science. p. 414. ISBN 0-8153-4093-1. ^ Hellman, Samuel; Mauch, P.M. Ed. (1999). Hodgkin's Disease. Chapter 1: Lippincott Williams & Wilkins. p. 5. ISBN 0-7817-1502-4. ^ Wagman LD. "Principles of Surgical Oncology" in Pazdur R, Wagman LD, Camphausen KA, Hoskins WJ (Eds) Cancer Management: A Multidisciplinary Approach. 11 ed. 2008. ^ a b ed. by Elaine S. Jaffe .... (2001). Pathology and Genetics of Haemo (World Health Organization Classification of tumors S.). Oxford Univ Pr. ISBN 92-832-2411-6. ^ Lennert, Karl; Feller, Alfred C.; Jacques Diebold; M. Paulli; A. Le Tourneau (2002). Histopathology of Non-Hodgkin's Lymphomas (Based on the Updated Kiel Classification). Berlin: Springer. pp. 2. ISBN 3-540-63801-6. ^ www.emedicine.com on Lymphoma, Non-Hodgkin ^ Clarke CA, Glaser SL, Dorfman RF, Bracci PM, Eberle E, Holly EA (January 2004). "Expert review of non-Hodgkin's lymphomas in a population-based cancer registry: reliability of diagnosis and subtype classifications". Cancer Epidemiol. Biomarkers Prev. 13 (1): 138–43. doi:10.1158/1055-9965.EPI-03-0250. PMID 14744745. ^ http://lymphoma.about.com/od/symptoms/tp/warningsigns.htm ^ "WHO Disease and injury country estimates". World Health Organization. 2009. Retrieved Nov. 11, 2009. ^ Horner MJ, Ries LAG, Krapcho M, Neyman N, et al. (eds).. "SEER Cancer Statistics Review, 1975–2006". Surveillance Epidemiology and End Results (SEER). Bethesda, MD: National Cancer Institute. Retrieved 03 November 2009. "Table 1.4: Age-Adjusted SEER Incidence and U.S. Death Rates and 5-Year Relative Survival Rates By Primary Cancer Site, Sex and Time Period" [edit]External links Timeline of discovery and treatment of Hodgkin's Lymphoma US lymphoma statistics from the United States National Cancer Institute Hodgkin Lymphoma and UK Non-Hodgkin Lymphoma statistics from the UK Latest news and research on Lymphoma Lymphoma Imaging Appearance - Chest Radiography [hide] v • d • e Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208) Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590-9739, 9800-9839) B cell (lymphoma, leukemia) (most CD19, CD20) By development/ marker TdT+ ALL (Precursor B acute lymphoblastic leukemia/lymphoma) CD5+ naive B cell (CLL/SLL) mantle zone (Mantle cell) CD22+ Prolymphocytic · CD11c (Hairy cell leukemia) CD79a+ germinal center/follicular B cell (Follicular, Burkitt's, GCB-DLBCL) marginal zone/marginal-zone B cell (Splenic marginal zone, MALT, Nodal marginal zone) RS (CD15+,CD30+) Classic Hodgkin's lymphoma (Nodular sclerosis) · CD20 (Nodular lymphocyte predominant Hodgkin's lymphoma) PCDs/PP (CD38+/CD138+) see immunoproliferative immunoglobulin disorders By infection KSHV (Primary effusion) · EBV (Lymphomatoid granulomatosis, Post-transplant lymphoproliferative disorder) · HIV (AIDS-related lymphoma) · Helicobacter pylori (MALT lymphoma) T/NK T cell (lymphoma, leukemia) (most CD3, CD4, CD8) By development/ marker TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma) prolymphocyte (Prolymphocytic) CD30+ (Anaplastic large cell, Lymphomatoid papulosis) By location/peripheral Cutaneous (Mycosis fungoides, Sézary's disease) · Hepatosplenic · Angioimmunoblastic · Enteropathy-associated T-cell lymphoma By infection HTLV-1 (Adult T-cell leukemia/lymphoma) NK cell/ (most CD56) Aggressive NK-cell leukemia · Blastic NK cell lymphoma T or NK EBV (Extranodal NK-T-cell lymphoma) · Large granular lymphocytic leukemia Lymphoid+myeloid Acute biphenotypic leukaemia Lymphocytosis Lymphoproliferative disorders (X-linked lymphoproliferative disease, Autoimmune lymphoproliferative syndrome) · Leukemoid reaction · Pseudolymphoma lymphocyte navs: cells/physio, immunodeficiency/immunoproliferative immunoglobulin/neoplasia, proc Categories: Blood disorders | Types of cancer | Deaths from lymphoma | Hematopathology | Lymphoma article discussion edit this page history Try Beta Log in / create account navigation Main page Contents Featured content Current events Random article search interaction About Wikipedia Community portal Recent changes Contact Wikipedia Donate to Wikipedia Help toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page languages العربية বাংলা Български Català Deutsch Español Euskara Français Gaeilge Hrvatski Bahasa Indonesia Interlingua Italiano עברית Lietuviù Nederlands 日本語 Norsk (bokmål) Polski Português Русский Српски / Srpski Suomi Svenska Türkçe Українська 中文 This page was last modified on 25 March 2010 at 23:17. 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