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Lymphoma

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This article is about lymphoma in humans. For the disease in dogs, cats, and ferrets, see lymphoma in animals.

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Lymphoma
SpecialtyHematology, oncology Edit this on Wikidata


Lymphoma is a cancer that begins in the lymphatic cells 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]

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]

Working Formulation and Non-Hodgkin lymphoma

Main article: Working Formulation

The 1982 Working Formulation is a classification of non-Hodgkin lymphoma. It excluded the Hodgkin's lymphomas and divided the remaining lymphomas into four grades (Low, Intermediate, High, and Miscellaneous) related to prognosis, with some further subdivisions based on the size and shape of affected cells. This purely histological classification 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 widely accepted a the time of its publication but is now considered obsolete. [6] It was superseded by subsequent classifications (see below) but it is still used by cancer agencies for compilation of lymphoma statistics and historical comparisons.

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.[7] REAL has been superseded by the WHO classification.

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.

Mature B cell neoplasms

File:Lymphoma microarray.jpg
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.

Mature T cell and natural killer (NK) cell neoplasms

Hodgkin lymphoma

Immunodeficiency-associated lymphoproliferative disorders

Other classification systems

Symptoms

Diagnosis, etiology, staging, prognosis, and treatment

These depend on the specific form of lymphoma.[9] For some forms lymphoma, watchful waiting is often the initial course of action.[10] If a low-grade lymphoma is becoming symptomatic, radiotherapy or chemotherapy are the treatments of choice, although they do not cure the lymphoma, they can alleviate the symptoms, particularly painful lymphadenopathy. Patients with these types lymphoma can live near-normal lifespans, but the disease is incurable. Treatment of some other, more aggressive, forms of lymphoma can result in a cure in the majority of cases, however, the prognosis for patients with a poor response to therapy is worse.[11] Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP regimen. Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized.[12] Advanced Hodgkins disease requires systemic chemotherapy, sometimes combined with radiotherapy.[13] See the articles on the corresponding form of lymphoma for further information.

Epidemiology

Age-standardized death from lymphomas and multiple myeloma per 100,000 inhabitants in 2004.[14]
  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.[15]

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.

See also

References

  1. ^ a b Parham, Peter (2005). The immune system. New York: Garland Science. p. 414. ISBN 0-8153-4093-1.
  2. ^ Hellman, Samuel (1999). Hodgkin's Disease. Chapter 1: Lippincott Williams & Wilkins. p. 5. ISBN 0-7817-1502-4. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)CS1 maint: location (link)
  3. ^ Wagman LD. "Principles of Surgical Oncology" in Pazdur R, Wagman LD, Camphausen KA, Hoskins WJ (Eds) Cancer Management: A Multidisciplinary Approach. 11 ed. 2008.
  4. ^ 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. {{cite book}}: |author= has generic name (help)
  5. ^ 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. p. 2. ISBN 3-540-63801-6.{{cite book}}: CS1 maint: multiple names: authors list (link)
  6. ^ Clarke CA, Glaser SL, Dorfman RF, Bracci PM, Eberle E, Holly EA (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. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  7. ^ www.emedicine.com on Lymphoma, Non-Hodgkin
  8. ^ http://lymphoma.about.com/od/symptoms/tp/warningsigns.htm
  9. ^ Sweetenham JW (2009). "Treatment of lymphoblastic lymphoma in adults". Oncology (Williston Park, N.Y.). 23 (12): 1015–20. PMID 20017283. {{cite journal}}: Unknown parameter |month= ignored (help)
  10. ^ Elphee EE (2008). "Understanding the concept of uncertainty in patients with indolent lymphoma". Oncol Nurs Forum. 35 (3): 449–54. doi:10.1188/08.ONF.449-454. PMID 18467294. {{cite journal}}: Unknown parameter |month= ignored (help)
  11. ^ Bernstein SH, Burack WR (2009). "The incidence, natural history, biology, and treatment of transformed lymphomas". Hematology Am Soc Hematol Educ Program: 532–41. doi:10.1182/asheducation-2009.1.532. PMID 20008238.
  12. ^ Martin NE, Ng AK (2009). "Good things come in small packages: low-dose radiation as palliation for indolent non-Hodgkin lymphomas". Leuk. Lymphoma. 50 (11): 1765–72. doi:10.3109/10428190903186510. PMID 19883306. {{cite journal}}: Unknown parameter |month= ignored (help)
  13. ^ Kuruvilla J (2009). "Standard therapy of advanced Hodgkin lymphoma". Hematology Am Soc Hematol Educ Program: 497–506. doi:10.1182/asheducation-2009.1.497. PMID 20008235.
  14. ^ "WHO Disease and injury country estimates". World Health Organization. 2009. Retrieved Nov. 11, 2009. {{cite web}}: Check date values in: |accessdate= (help)
  15. ^ 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 {{cite web}}: Check date values in: |accessdate= (help)CS1 maint: multiple names: authors list (link)
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