Lymphoma is a
cancerCancer is a class of diseases in which a group of cells display uncontrolled growth , invasion , and sometimes metastasis...
that begins in the
lymphocyteA lymphocyte is a type of white blood cell in the vertebrate immune system.By their appearance under the light microscope, there are two broad categories of lymphocytes, namely the large granular lymphocytes and the small lymphocytes. Functionally distinct subsets of lymphocytes correlate with...
s of the
immune systemAn immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumour cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
and presents as a solid
tumorA tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells . Tumor is not synonymous with cancer. A tumor can be benign, pre-malignant or malignant, whereas cancer is by definition malignant....
of lymphoid cells. They often originate like balls in
lymph nodeA lymph node is an organ consisting of many types of cells, and is a part of the lymphatic system. Lymph nodes are found all through the body, and act as filters or traps for foreign particles. They contain white blood cells that use oxygen to process. Thus they are important in the proper...
s, 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 marrowBone marrow is the flexible tissue found in the hollow interior of bones. In adults, marrow in large bones produces new blood cells. It constitutes 4% of total body weight, i.e...
(where blood cells are generated in a process termed haematopoesis) and do not usually form static tumours. There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases called
hematological neoplasmsHematological malignancies are the types of cancer that affect blood, bone marrow, and lymph nodes. As the three are intimately connected through the immune system, a disease affecting one of the three will often affect the others as well: although lymphoma is technically a disease of the lymph...
.
Thomas HodgkinThomas Hodgkin was a British physician, considered one of the most prominent pathologists of his time and a pioneer in preventive medicine. He is now best known for the first account of Hodgkin's disease, a form of lymphoma and blood disease, in 1832...
published in 1832 the first description of lymphoma, specifically of the form named after him,
Hodgkin's lymphomaHodgkin's lymphoma, also known as Hodgkin's disease, is a type of lymphoma...
. Since then many other forms of lymphoma have been described, grouped under several proposed classifications. The 1982
Working formulationThe Working Formulation is a classification of Non-Hodgkin Lymphoma published in 1982. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2001, but is still used by cancer agencies for compilation of lymphoma statistics.Low Grade*Malignant Lymphoma,...
classification became very popular. It introduced the category
non-Hodgkin lymphomaThe Non-Hodgkin lymphomas are a diverse group of hematologic cancers which encompass any lymphoma other than Hodgkin's Lymphoma.Lymphoma is a type of cancer derived from lymphocytes, a type of white blood cell. Many subtypes of non-Hodgkin lymphoma have been described...
(NHL), itself divided into 16 different diseases. However, since 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
WHOThe World Health Organization is a specialized agency of the United Nations that acts as a coordinating authority on international public health...
(2001) lists 43 different forms of lymphoma divided in four broad groups.
Some forms of lymphoma are indolent (e.g. small lymphocytic lymphoma), compatible with a long life even without treatment, whereas other forms are aggressive (e.g.
Burkitt's lymphomaBurkitt lymphoma is a cancer of the lymphatic system . It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.-Genetics:Almost by definition, Burkitt's lymphoma is associated with c-myc gene translocation...
), causing rapid deterioration and death. The
prognosisPrognosis is a medical term to describe the likely outcome of an illness. When applied to large populations, prognostic estimates can be very accurate: for example the statement "45% of patients with severe septic shock will die within 28 days" can be made with some confidence, because previous...
therefore depends on the correct classification of the disease, established by a
pathologistPathology is the study and diagnosis of disease through examination of organs, tissues, bodily fluids, and whole bodies...
after examination of a
biopsyA biopsy is a medical test involving the removal of cells or tissues for examination. It is the removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
.
Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of
BB cells are lymphocytes that play a large role in the humoral immune response . The principal functions of B cells are to make antibodies against antigens, perform the role of Antigen Presenting Cells and eventually develop into memory B cells after activation by antigen interaction...
,
T'T cells' belong to a group of white blood cells known as lymphocytes, and play a central role in cell-mediated immunity. They can be distinguished from other lymphocyte types, such as B cells and natural killer cells by the presence of a special receptor on their cell surface called T cell receptors...
or NK cell lineage. True
histiocyticA histiocyte is a cell that is part of the mononuclear phagocytic system . The mononuclear phagocytic system is part of the organism's immune system...
malignancies are rare and are classified as
sarcomaA sarcoma is a cancer of the connective tissue resulting in mesoderm proliferation.This is in contrast to carcinomas, which are of epithelial origin...
s.
Prevalence
According to the
U.S. National Institutes of HealthThe National Institutes of Health is an agency of the United States Department of Health and Human Services and is the primary agency of the United States government responsible for biomedical and health-related research. It consists of 27 separate institutes and centers which includes the Office...
, 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.
Kiel classification
As an alternative to the American Lukes-Butler classification, in the early 1970s, Karl Lennert proposed a new system of classifying lymphomas based on cellular
morphologyIn biology morphology is the form, structure and configuration of an organism.This includes aspects of the outward appearance as well as the form and structure of the internal parts like bones and organs...
and their relationship to cells of the normal peripheral lymphoid system.
REAL classification
In the mid 1990s, the
Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic NHL entities.
WHO classification
The
WHO Classification, published in 2001 and updated in 2008, 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 tumour) and defining phenotypic, molecular or cytogenetic characteristics. There are three large groups: the
B cellB cells are lymphocytes that play a large role in the humoral immune response . The principal functions of B cells are to make antibodies against antigens, perform the role of Antigen Presenting Cells and eventually develop into memory B cells after activation by antigen interaction...
,
T cell'T cells' belong to a group of white blood cells known as lymphocytes, and play a central role in cell-mediated immunity. They can be distinguished from other lymphocyte types, such as B cells and natural killer cells by the presence of a special receptor on their cell surface called T cell receptors...
, and
natural killer cellNatural killer cells are a type of cytotoxic lymphocyte that constitute a major component of the innate immune system. NK cells play a major role in the rejection of tumors and cells infected by viruses...
tumours. Other less common groups, are also recognised.
Hodgkin's lymphomaHodgkin's lymphoma, also known as Hodgkin's disease, is a type of lymphoma...
, although considered separately within the
WHO*Who is an English language interrogative pronoun.-In fiction:*Who? , a 1958 novel by science fiction author Algis Budrys, turned into a film with the same title in 1973*Doctor Who, a British science fiction television series...
(and preceding) classifications, is now recognised as being a tumour of, albeit markedly abnormal, lymphocytes of mature
B cellB cells are lymphocytes that play a large role in the humoral immune response . The principal functions of B cells are to make antibodies against antigens, perform the role of Antigen Presenting Cells and eventually develop into memory B cells after activation by antigen interaction...
lineage.
Mature B cell neoplasms
- Chronic lymphocytic leukemia
B-cell chronic lymphocytic leukemia , also known as chronic lymphoid leukemia , is the most common type of leukemia. Leukemias are abnormal and malignant neoplastic proliferations of the white blood cells . CLL involves a particular subtype of white blood cells, which is a lymphocyte called a B...
/Small lymphocytic lymphoma
- B-cell prolymphocytic leukemia
B-cell prolymphocytic leukemia is a more aggressive, but still treatable, form of leukemia. The malignant B cells are larger than average. The name is commonly abbreviated B-PLL.It can involve deletions from chromosome 11 and chromosome 13....
- Lymphoplasmacytic lymphoma (such as Waldenström macroglobulinemia
Waldenström's macroglobulinemia is cancer involving a subtype of white blood cells called lymphocytes. The main attributing antibody is IgM. It is a type of lymphoproliferative disease, and shares clinical characteristics with the indolent non-Hodgkin lymphomas.It is named after the Swedish...
)
- Splenic marginal zone lymphoma
Splenic marginal zone lymphoma is a lymphoma made up of B-cells that replace the normal architecture of the white pulp of the spleen. The neoplastic cells are both small lymphocytes and larger, transformed blasts, and they invade the mantle zone of splenic follicles and erode the marginal zone,...
- Plasma cell
Plasma cells, also called plasma B cells, plasmocytes, and effector B cells, are white blood cells that produce large volumes of antibodies. They are transported by the blood plasma and the lymphatic system...
neoplasms:
- Plasma cell myeloma
- Plasmacytoma
Plasmacytoma refers to a malignant plasma cell tumor growing within soft tissue or within the skeleton. The skeletal forms usually have other occult tumors and frequently disseminate to multiple myeloma over the course of 5–10 years. The soft tissue forms most often occur in the upper respiratory...
- Monoclonal immunoglobulin deposition diseases
- Heavy chain diseases
- Extranodal marginal zone B cell lymphoma, also called MALT lymphoma
MALT lymphoma is a form of lymphoma involving the mucosa-associated lymphoid tissue , frequently of the stomach, but virtually any mucosal site can be afflicted...
- Nodal marginal zone B cell lymphoma
Nodal marginal zone B cell lymphoma is a form of marginal-zone lymphoma that can produce colonization of the follicles....
(NMZL)
- Follicular lymphoma
Follicular lymphoma is the second most common of the indolent non-Hodgkin's lymphomas. It is defined as a lymphoma of follicle center B-cells , which has at least a partially follicular pattern...
- Mantle cell lymphoma
Mantle cell lymphoma is one of the rarer of the non-Hodgkin's lymphomas , comprising about 6% of NHL cases. There are only about 15,000 patients presently in the U.S. While it is difficult to treat and seldom considered cured, investigations into better treatments are actively pursued worldwide...
- Diffuse large B cell lymphoma
Diffuse large B-cell lymphoma is a type of aggressive lymphoma. It accounts for approximately 40% of lymphomas among adults. Of all cancers involving the same class of blood cell, 31% of cases are DLBL.-Classification:...
- Mediastinal (thymic) large B cell lymphoma
- Intravascular large B cell lymphoma
- Primary effusion lymphoma
-Causes:It is caused by Kaposi's sarcoma-associated herpesvirus , also known as human herpesvirus 8 . In most cases, the lymphoma cells are also infected with Epstein Barr virus ....
- Burkitt lymphoma/leukemia
Burkitt lymphoma is a cancer of the lymphatic system . It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.-Genetics:Almost by definition, Burkitt's lymphoma is associated with c-myc gene translocation...
Mature T cell and natural killer (NK) cell neoplasms
- T cell prolymphocytic leukemia
T-cell-prolymphocytic leukemia is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement. T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic...
- T cell large granular lymphocytic leukemia
Large granular lymphocytic leukemia is a disease that exhibits an unexplained, chronic elevation in large granular lymphocytes in the peripheral blood....
- 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
Mycosis fungoides , is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time....
/ Sezary syndrome
- Primary cutaneous CD30-positive T cell lymphoproliferative disorders
- Primary cutaneous anaplastic large cell lymphoma
- Lymphomatoid papulosis
Lymphomatoid papulosis is a rare skin disorder. The overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population.This rare condition has only been studied in depth since 1968.It can evolve into lymphoma....
- Angioimmunoblastic T cell lymphoma
- Peripheral T cell lymphoma, unspecified
- Anaplastic large cell lymphoma
Anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma that features in the World Health Organisation classification of lymphomas.Its name derives from anaplasia and large cell lymphoma.-Signs and symptoms:...
Hodgkin lymphoma
- Classical Hodgkin lymphomas:
- Nodular sclerosis
Nodular sclerosis is a form of Hodgkin's lymphoma which is the most common subtype of HL in developed countries. It usually affects females more than males and has a median age of onset at ~28 years...
- Mixed cellularity
- Lymphocyte-rich
- Lymphocyte depleted or not depleted
- Nodular lymphocyte-predominant Hodgkin lymphoma
Immunodeficiency-associated lymphoproliferative disorders
- Associated with a primary immune disorder
- Associated with the Human Immunodeficiency Virus (HIV
Human immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections. Infection with HIV occurs by the transfer of blood, semen, vaginal fluid,...
)
- Post-transplant
- Associated with Methotrexate therapy
Working formulation and Non-Hodgkin lymphoma
The 1982
Working FormulationThe Working Formulation is a classification of Non-Hodgkin Lymphoma published in 1982. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2001, but is still used by cancer agencies for compilation of lymphoma statistics.Low Grade*Malignant Lymphoma,...
is a classification of
Non-Hodgkin LymphomaThe Non-Hodgkin lymphomas are a diverse group of hematologic cancers which encompass any lymphoma other than Hodgkin's Lymphoma.Lymphoma is a type of cancer derived from lymphocytes, a type of white blood cell. Many subtypes of non-Hodgkin lymphoma have been described...
. It has since been replaced by other lymphoma classifications, the latest published by the
WHOThe World Health Organization is a specialized agency of the United Nations that acts as a coordinating authority on international public health...
in 2001 (updated in September 2008), but is still used by cancer agencies for compilation of lymphoma statistics.
Other classification systems
- ICD-O (codes 9590-9999, details at http://web.archive.org/web/20040627090029/http://www.cog.ufl.edu/publ/apps/icdo/icdo_morph.txt) (archive
The Internet Archive is a nonprofit organization dedicated to building and maintaining a free and openly accessible online digital library, including an archive of the World Wide Web....
link, was deadA dead link is a link on the World Wide Web that points to a web page or server that is permanently unavailable. The most common result of a dead link is a 404 error, which indicates that the web server responded, but the specific page could not be found...
)
- ICD-10
The International Statistical Classification of Diseases and Related Health Problems provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings,...
(codes C81-C96, details at http://www3.who.int/icd/vol1htm2003/fr-icd.htm?gc81.htm+)
Diagnosis, etiology, staging, prognosis, and treatment
See the article of the corresponding form of lymphoma.
Association of medications with lymphoma
Inflammatory Bowel DiseaseIn medicine, inflammatory bowel disease is a group of inflammatory conditions of the colon and small intestine. The major types of IBD are Crohn's disease and ulcerative colitis..-Forms:...
(IBD) patients treated with the cancer medications, 6-
MercaptopurineMercaptopurine is an immunosuppressive drug.It is a thiopurine.-Uses:It is used to treat leukemia...
(Purinethol) and
AzathioprineAzathioprine is a drug that suppresses the immune system.Azathioprine is used in organ transplantation and autoimmune disease. Some of the autoimmune diseases are rheumatoid arthritis, pemphigus, Inflammatory Bowel Disease , multiple sclerosis, autoimmune hepatitis and restrictive lung...
(Imuran) are at a greater risk for developing lymphoma. This risk is 4 times higher than the general population.
See also
- Hodgkin's lymphoma
Hodgkin's lymphoma, also known as Hodgkin's disease, is a type of lymphoma...
- Non-Hodgkin's lymphoma
- Follicular lymphoma
Follicular lymphoma is the second most common of the indolent non-Hodgkin's lymphomas. It is defined as a lymphoma of follicle center B-cells , which has at least a partially follicular pattern...
- Burkitt's lymphoma
Burkitt lymphoma is a cancer of the lymphatic system . It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.-Genetics:Almost by definition, Burkitt's lymphoma is associated with c-myc gene translocation...
- Mantle cell lymphoma
Mantle cell lymphoma is one of the rarer of the non-Hodgkin's lymphomas , comprising about 6% of NHL cases. There are only about 15,000 patients presently in the U.S. While it is difficult to treat and seldom considered cured, investigations into better treatments are actively pursued worldwide...
- Gastric lymphoma
Primary gastric lymphoma is an uncommon condition, accounting for less than 15% of gastric malignancies and about 2% of all lymphomas. However, the stomach is a very common extranodal site for lymphomas...
- Cutaneous T Cell lymphoma
Cutaneous T cell lymphoma is a class of non-Hodgkin's lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin's lymphomas , CTCL is caused by a mutation of T cells...
- Mycosis fungoides
Mycosis fungoides , is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time....
- Anaplastic large cell lymphoma
Anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma that features in the World Health Organisation classification of lymphomas.Its name derives from anaplasia and large cell lymphoma.-Signs and symptoms:...
- MALT lymphoma
MALT lymphoma is a form of lymphoma involving the mucosa-associated lymphoid tissue , frequently of the stomach, but virtually any mucosal site can be afflicted...
- Primary central nervous system lymphoma
A primary CNS lymphoma is a primary intracranial tumor appearing primarily in patients with severe immunosuppression . PCNSLs represent around 20% of all cases of lymphomas in HIV infections...
- BCP-1 cells
BCP-1 cells are a clonal lymphoma cell line. They were derived from the peripheral blood mononuclear cells of a HIV seronegative patient with a body cavity based primary effusion lymphoma . BCP-1 cells are positive for KSHV, but negative for EBV...
- Ann Arbor staging
Ann Arbor staging is the staging system for lymphomas, both in Hodgkin's lymphoma and Non-Hodgkin lymphoma . It was initially developed for Hodgkin's, but has some use in NHL...
- International Prognostic Index
The International Prognostic Index is a clinical tool developed by oncologists to aid in predicting the prognosis of patients with aggressive non-Hodgkin's lymphoma...
External links