Lymphoma
Encyclopedia
Lymphoma is a cancer
in the lymphatic cells of the immune system
. Typically, lymphomas present as a solid tumor
of lymphoid cells. Treatment might involve 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. These malignant cells often originate in lymph node
s, presenting as an enlargement of the node (a tumor). It can also affect other organs in which case it is referred to as extranodal lymphoma. Extranodal sites include the skin, brain, bowels and bone. 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. 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
. 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
(2008) lists 70 different forms of lymphoma divided in four broad groups.
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 sarcoma
s.
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.
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 diagnosis and classification of the disease, which is established after examination of a biopsy
by a pathologist
(usually a hematopathologist
).
is a classification of non-Hodgkin lymphoma
. It excluded the Hodgkin 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 lymphoma
s or B-cell lymphoma
s.See here
It was widely accepted at the time of its publication but is now obsolete. It was superseded by subsequent classifications (see below) but it is still used by cancer agencies for compilation of lymphoma statistics and historical rare comparisons.
. REAL has been superseded by the WHO classification.
REAL & WHO
•B-cell neoplasms
–precursor
–mature
•T-cell neoplasms
–precursor
–mature
•Hodgkin lymphomaNon-HodgkinLymphomas
, T cell
, and natural killer cell
tumors. Other less common groups, are also recognized. Hodgkin's lymphoma, although considered separately within the World Health Organization
(and preceding) classifications, is now recognized as being a tumor of, albeit markedly abnormal, lymphocytes of mature B cell lineage.
These depend on the specific form of lymphoma. For some forms of 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; although they do not cure the lymphoma, they can alleviate the symptoms, particularly painful lymphadenopathy
. Patients with these types of 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, but the prognosis for patients with a poor response to therapy is worse. Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP
or RCHOP regimen. Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized. Advanced Hodgkin disease requires systemic chemotherapy, sometimes combined with radiotherapy. See the articles on the corresponding form of lymphoma for further information.
, 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.
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.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
in the lymphatic cells of the immune system
Immune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
. Typically, lymphomas present as a solid tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
of lymphoid cells. Treatment might involve chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage of the disease. These malignant cells often originate in lymph node
Lymph node
A lymph node is a small ball or an oval-shaped organ of the immune system, distributed widely throughout the body including the armpit and stomach/gut and linked by lymphatic vessels. Lymph nodes are garrisons of B, T, and other immune cells. Lymph nodes are found all through the body, and act as...
s, presenting as an enlargement of the node (a tumor). It can also affect other organs in which case it is referred to as extranodal lymphoma. Extranodal sites include the skin, brain, bowels and bone. Lymphomas are closely related to lymphoid leukemias, which also originate in lymphocytes but typically involve only circulating blood and the bone marrow
Bone marrow
Bone marrow is the flexible tissue found in the interior of bones. In humans, bone marrow in large bones produces new blood cells. On average, bone marrow constitutes 4% of the total body mass of humans; in adults weighing 65 kg , bone marrow accounts for approximately 2.6 kg...
(where blood cells are generated in a process termed haematopoesis) and do not usually form static tumors. There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases called hematological neoplasms
Hematological malignancy
Hematological 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 Hodgkin
Thomas Hodgkin
Thomas 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 the first description of lymphoma in 1832, specifically of the form named after him, Hodgkin's lymphoma
Hodgkin's lymphoma
Hodgkin's lymphoma, previously known as Hodgkin's disease, is a type of lymphoma, which is a cancer originating from white blood cells called lymphocytes...
. Since then, many other forms of lymphoma have been described, grouped under several proposed classifications. The 1982 Working formulation
Working Formulation
The Working Formulation is an obsolete classification of non-Hodgkin lymphomas published in 1982. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2008, but is still used by cancer agencies for compilation of lymphoma statistics.Low Grade*Malignant...
classification became very popular. It introduced the category non-Hodgkin lymphoma
Non-Hodgkin lymphoma
The non-Hodgkin lymphomas are a diverse group of blood cancers that include any kind of lymphoma except Hodgkin's lymphomas. Types of NHL vary significantly in their severity, from indolent to very aggressive....
(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
World Health Organization
The World Health Organization is a specialized agency of the United Nations that acts as a coordinating authority on international public health. Established on 7 April 1948, with headquarters in Geneva, Switzerland, the agency inherited the mandate and resources of its predecessor, the Health...
(2008) lists 70 different forms of lymphoma divided in four broad groups.
Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B
B cell
B 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 cell
T cells or T lymphocytes 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 lymphocytes, such as B cells and natural killer cells , by the presence of a T cell receptor on the cell surface. They are...
or NK cell lineage. True histiocytic
Histiocyte
A histiocyte is a cell that is part of the mononuclear phagocyte system . The mononuclear phagocytic system is part of the organism's immune system...
malignancies are rare and are classified as sarcoma
Sarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
s.
Classification
A number of various classification systems exist for lymphoma.As an alternative to the American Lukes-Butler classification, in the early 1970s, Karl Lennert of Kiel
Kiel
Kiel is the capital and most populous city in the northern German state of Schleswig-Holstein, with a population of 238,049 .Kiel is approximately north of Hamburg. Due to its geographic location in the north of Germany, the southeast of the Jutland peninsula, and the southwestern shore of the...
, Germany, proposed a new system of classifying lymphomas based on cellular morphology
Morphology (biology)
In biology, morphology is a branch of bioscience dealing with the study of the form and structure of organisms and their specific structural features....
and their relationship to cells of the normal peripheral lymphoid system.
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
Burkitt's lymphoma
Burkitt's lymphoma is a cancer of the lymphatic system...
), causing rapid deterioration and death. However, most of the aggressive lymphomas respond well to treatment and are curable. The prognosis
Prognosis
Prognosis is a medical term to describe the likely outcome of an illness.When applied to large statistical 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...
therefore depends on the correct diagnosis and classification of the disease, which is established after examination of a biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical 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...
by a pathologist
Pathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
(usually a hematopathologist
Hematopathology
Hematopathology is the branch of pathology which studies diseases of hematopoietic cells . In the United States, hematopathology is a board certified subspecialty practiced by those physicians who have completed general pathology residency and additional fellowship training in...
).
Working Formulation and non-Hodgkin lymphoma
The 1996 Working FormulationWorking Formulation
The Working Formulation is an obsolete classification of non-Hodgkin lymphomas published in 1982. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2008, but is still used by cancer agencies for compilation of lymphoma statistics.Low Grade*Malignant...
is a classification of non-Hodgkin lymphoma
Non-Hodgkin lymphoma
The non-Hodgkin lymphomas are a diverse group of blood cancers that include any kind of lymphoma except Hodgkin's lymphomas. Types of NHL vary significantly in their severity, from indolent to very aggressive....
. It excluded the Hodgkin 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 lymphoma
T-cell lymphoma
The T-cell lymphomas are the four types of lymphoma that affect T cells. These account for perhaps one in ten cases of non-Hodgkin lymphoma.They can be associated with Epstein Barr virus and Human T-cell leukemia virus-1.-Types:The four classes are:...
s or B-cell lymphoma
B-cell lymphoma
The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph glands. They develop more frequently in older adults and in immunocompromised individuals ....
s.See here
It was widely accepted at the time of its publication but is now obsolete. It was superseded by subsequent classifications (see below) but it is still used by cancer agencies for compilation of lymphoma statistics and historical rare 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 lymphomaHodgkin's lymphoma
Hodgkin's lymphoma, previously known as Hodgkin's disease, is a type of lymphoma, which is a cancer originating from white blood cells called lymphocytes...
. REAL has been superseded by the WHO classification.
REAL & WHO
•B-cell neoplasms
–precursor
–mature
•T-cell neoplasms
–precursor
–mature
•Hodgkin lymphomaNon-HodgkinLymphomas
World Health Organization (WHO)
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 tumor) and defining phenotypic, molecular or cytogenetic characteristics. There are three large groups: the B cellB cell
B 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 cell
T cells or T lymphocytes 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 lymphocytes, such as B cells and natural killer cells , by the presence of a T cell receptor on the cell surface. They are...
, and natural killer cell
Natural killer cell
Natural 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...
tumors. Other less common groups, are also recognized. Hodgkin's lymphoma, although considered separately within the World Health Organization
World Health Organization
The World Health Organization is a specialized agency of the United Nations that acts as a coordinating authority on international public health. Established on 7 April 1948, with headquarters in Geneva, Switzerland, the agency inherited the mandate and resources of its predecessor, the Health...
(and preceding) classifications, is now recognized as being a tumor of, albeit markedly abnormal, lymphocytes of mature B cell lineage.
Mature B cell neoplasms
- Chronic lymphocytic leukemiaChronic lymphocytic leukemiaB-cell chronic lymphocytic leukemia , also known as chronic lymphoid leukemia , is the most common type of leukemia. Leukemias are cancers of the white blood cells . CLL affects B cell lymphocytes. B cells originate in the bone marrow, develop in the lymph nodes, and normally fight infection by...
/Small lymphocytic lymphoma - B-cell prolymphocytic leukemiaB-cell prolymphocytic leukemiaB-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 macroglobulinemiaWaldenström macroglobulinemiaWaldenström's macroglobulinemia is cancer involving a subtype of white blood cells called lymphocytes. The main attributing antibody is Immunoglobulin M . WM is an "indolent lymphoma,"...
) - Splenic marginal zone lymphomaSplenic marginal zone lymphomaSplenic 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 cellPlasma cellPlasma cells, also called plasma B cells, plasmocytes, and effector B cells, are white blood cells which produce large volumes of antibodies. They are transported by the blood plasma and the lymphatic system...
neoplasms:- Plasma cell myeloma
- PlasmacytomaPlasmacytomaPlasmacytoma 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 lymphomaMALT lymphomaMALT 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 lymphomaNodal marginal zone B cell lymphomaNodal marginal zone B cell lymphoma is a form of marginal-zone lymphoma that can produce colonization of the follicles....
(NMZL) - Follicular lymphomaFollicular lymphomaFollicular lymphoma is the most common of the indolent non-Hodgkin's lymphomas, and the second most common form of non-Hodgkin's lymphomas overall. It is defined as a lymphoma of follicle center B-cells , which has at least a partially follicular pattern...
- Mantle cell lymphomaMantle cell lymphomaMantle cell lymphoma is one of the rarest 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 lymphomaDiffuse large B cell lymphomaDiffuse large B-cell lymphoma is a type of aggressive non-Hodgkin lymphoma. It accounts for approximately 40% of lymphomas among adults. The median age at diagnosis is 70 years, but it also occurs in children and young adults...
- Mediastinal (thymic) large B cell lymphoma
- Intravascular large B cell lymphoma
- Primary effusion lymphomaPrimary 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/leukemiaBurkitt's lymphomaBurkitt's lymphoma is a cancer of the lymphatic system...
Mature T cell and natural killer (NK) cell neoplasms
- T cell prolymphocytic leukemiaT-cell prolymphocytic leukemiaT-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 leukemiaT-cell large granular lymphocyte leukemiaLarge 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 lymphomaBlastic NK cell lymphomaBlastic NK cell lymphoma is a type of lymphoma.It is positive for CD4 and CD56.It does not appear to be associated with Epstein Barr virus....
- Mycosis fungoidesMycosis fungoides-External links:* * *...
/ Sezary syndrome - Primary cutaneous CD30-positive T cell lymphoproliferative disorders
- Primary cutaneous anaplastic large cell lymphoma
- Lymphomatoid papulosisLymphomatoid papulosisLymphomatoid 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.-Presentation:...
- Angioimmunoblastic T cell lymphoma
- Peripheral T cell lymphoma, unspecified
- Anaplastic large cell lymphomaAnaplastic large cell lymphomaAnaplastic 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 sclerosisNodular sclerosisNodular sclerosis is a form of Hodgkin's lymphoma that is the most common subtype of HL in developed countries. It affects females and males equally and has a median age of onset at ~28 years...
- Mixed cellularity
- Lymphocyte-rich
- Lymphocyte depleted or not depleted
- Nodular sclerosis
- Nodular lymphocyte-predominant Hodgkin lymphoma
Immunodeficiency-associated lymphoproliferative disorders
- Associated with a primary immune disorder
- Associated with the Human Immunodeficiency Virus (HIVHIVHuman immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
) - Post-transplant
- Associated with methotrexateMethotrexateMethotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
therapy - Primary central nervous system lymphomaPrimary central nervous system lymphomaA primary central nervous system lymphoma , also known as microglioma and primary brain lymphoma, is a primary intracranial tumor appearing mostly in patients with severe immunosuppression . PCNSLs represent around 20% of all cases of lymphomas in HIV infections...
occurs most often in immuno-compromised patients, in particular those with AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
, but it can occur in the immunocompetent as well. It has a poor prognosis, particularly in those with AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
. Treatment can consist of corticosteroids, radiotherapy, and chemotherapyChemotherapyChemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
, often with methotrexateMethotrexateMethotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
.
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)
- ICD-10ICDThe International Statistical Classification of Diseases and Related Health Problems is a medical classification that provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or disease...
(codes C81-C96, details at http://www3.who.int/icd/vol1htm2003/fr-icd.htm?gc81.htm+)
Symptoms
- AnorexiaAnorexia (symptom)Anorexia is the decreased sensation of appetite...
- DyspneaDyspneaDyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
- Fatigue
- Fever of unknown originFever of unknown originFever of unknown origin , pyrexia of unknown origin or febris e causa ignota refers to a condition in which the patient has an elevated temperature but despite investigations by a physician no explanation has been found....
- LymphadenopathyLymphadenopathyLymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
- Night sweats
- Pruritus
- Weight lossWeight lossWeight loss, in the context of medicine, health or physical fitness, is a reduction of the total body mass, due to a mean loss of fluid, body fat or adipose tissue and/or lean mass, namely bone mineral deposits, muscle, tendon and other connective tissue...
Diagnosis, etiology, staging, prognosis, and treatment
| 5-year relative survival by stage at diagnosis | ||
|---|---|---|
| Stage at diagnosis | 5-year relative survival (%) | Percentage of cases (%) |
| Localized (confined to primary site) | 82.1 | 28 |
| Regional (spread to regional lymphnodes) | 77.5 | 19 |
| Distant (cancer has metastasized) | 59.9 | 45 |
| Unknown (unstaged) | 67.5 | 8 |
These depend on the specific form of lymphoma. For some forms of lymphoma, watchful waiting
Watchful waiting
Watchful waiting is an approach to a medical problem in which time is allowed to pass before medical intervention or therapy is used. During this time, repeated testing may be performed....
is often the initial course of action. 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
Lymphadenopathy
Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
. Patients with these types of 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, but the prognosis for patients with a poor response to therapy is worse. Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP
CHOP
CHOP is the acronym for a chemotherapy regimen used in the treatment of non-Hodgkin lymphoma. CHOP consists of:* Cyclophosphamide, an alkylating agent which damages DNA by binding to it and causing cross-links...
or RCHOP regimen. Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized. Advanced Hodgkin disease requires systemic chemotherapy, sometimes combined with radiotherapy. See the articles on the corresponding form of lymphoma for further information.
Epidemiology
Lymphoma is the most common form of hematological malignancyHematological malignancy
Hematological 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...
, 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.
According to the U.S. National Institutes of Health
National Institutes of Health
The National Institutes of Health are an agency of the United States Department of Health and Human Services and are the primary agency of the United States government responsible for biomedical and health-related research. Its science and engineering counterpart is the National Science Foundation...
, 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.
Comparison
Following is a comparison of the most common types of lymphoma:| Lymphoma type | Relative incidence Incidence (epidemiology) Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the... | Histopathology Histopathology Histopathology refers to the microscopic examination of tissue in order to study the manifestations of disease... | Cell markers | Overall 5-year survival Five-year survival rate The five-year survival rate is a term used in medicine for estimating the prognosis of a particular disease.Analysis performed against the Surveillance, Epidemiology, and End Results database facilitates calculation of Five-year survival rates.... | Other comments |
|---|---|---|---|---|---|
| Precursor T-cell leukemia/lymphoma | 40% of lymphomas in childhood. | Lymphoblast Lymphoblast Lymphoblasts are immature cells which typically differentiate to form mature lymphocytes. Normally lymphoblasts are found in the bone marrow, but in acute lymphoblastic leukemia , lymphoblasts proliferate uncontrollably and are found in large numbers in the peripheral blood.The size is between 10... s with irregular nuclear contours, condensed chromatin, small nucleoli and scant cytoplasm without granules. |
TdT Terminal Deoxynucleotidyl Transferase Terminal deoxynucleotidyl transferase , also known as DNA nucleotidylexotransferase or terminal transferase, is a specialized DNA polymerase expressed in immature, pre-B, pre-T lymphoid cells, and acute lymphoblastic leukemia/lymphoma cells... , CD2 CD2 CD2 is a cell adhesion molecule found on the surface of T cells and natural killer cells.It has also been called T-cell surface antigen T11/Leu-5, LFA-2, LFA-3 receptor, erythrocyte receptor and rosette receptor.... , CD7 CD7 CD7 is a human protein encoded by the gene.-External links:... |
It often presents as a mediastinal mass because of involvement of the thymus Thymus The thymus is a specialized organ of the immune system. The thymus produces and "educates" T-lymphocytes , which are critical cells of the adaptive immune system.... . It is highly associated with NOTCH1 NOTCH1 Notch homolog 1, translocation-associated , also known as NOTCH1, is a human gene encoding a single-pass transmembrane receptor.A deficiency can be associated with bicuspid aortic valve.... mutations. Most common in adolescent males. |
|
| Follicular lymphoma Follicular lymphoma Follicular lymphoma is the most common of the indolent non-Hodgkin's lymphomas, and the second most common form of non-Hodgkin's lymphomas overall. It is defined as a lymphoma of follicle center B-cells , which has at least a partially follicular pattern... |
40% of lymphomas in adults | Small "cleaved" cells (centrocyte Centrocyte A centrocyte generally refers to a B cell with a cleaved nucleus, as may appear in e.g. follicular lymphoma.It can also refer to a cell with a protoplasm that contains single and double granules of varying size stainable with hematoxylin, as seen in lesions of lichen planus, or a nondividing,... s) mixed with large activated cells (centroblasts). Usually nodular ("follicular") growth pattern |
CD10, surface Ig | 72–77% | Occurs in older adults. Usually involves lymph nodes, bone marrow and spleen. Associated with t(14;18) translocation Chromosomal translocation In genetics, a chromosome translocation is a chromosome abnormality caused by rearrangement of parts between nonhomologous chromosomes. A gene fusion may be created when the translocation joins two otherwise separated genes, the occurrence of which is common in cancer. It is detected on... overexpressing Bcl-2 Bcl-2 Bcl-2 is the founding member of the Bcl-2 family of apoptosis regulator proteins encoded by the BCL2 gene. Bcl-2 derives its name from B-cell lymphoma 2, as it is the second member of a range of proteins initially described in chromosomal translocations involving chromosomes 14 and 18 in... . Indolent |
| Diffuse large B cell lymphoma Diffuse large B cell lymphoma Diffuse large B-cell lymphoma is a type of aggressive non-Hodgkin lymphoma. It accounts for approximately 40% of lymphomas among adults. The median age at diagnosis is 70 years, but it also occurs in children and young adults... |
40 to 50% of lymphomas in adults | Variable. Most resemble B cells of large germinal centers. Diffuse growth pattern. | Variable expression of CD10 and surface Ig | 60% | Occurs in all ages, but most commonly in older adults. Often occurs outside lymph nodes. Aggressive. |
| Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma is one of the rarest 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... |
3 to 4% of lymphomas in adults | Lymphocytes of small to intermediate size growing in diffuse pattern | CD5 CD5 (protein) CD5 is a cluster of differentiation found on a subset of IgM-secreting B cells called B-1 cells, and also on T cells. B-1 cells have limited diversity of their B-cell receptor due to their lack of the enzyme terminal deoxynucleotidyl transferase and are potentially self-reactive... |
50% to 70% | Occurs mainly in adult males. Usually involves lymph nodes, bone marrow, spleen and GI tract Gastrointestinal tract The human gastrointestinal tract refers to the stomach and intestine, and sometimes to all the structures from the mouth to the anus. .... . Associated with t(11;14) translocation overexpressing cyclin D1 Cyclin D1 G1/S-specific cyclin-D1 is a protein that in humans is encoded by the CCND1 gene.Immunohistochemical staining of cyclin D1 antibodies is used to diagnose mantle cell lymphoma.-Interactions:... . Moderately aggressive. |
| B-cell chronic lymphocytic leukemia/lymphoma | 3 to 4 % of lymphomas in adults | Small resting lymphocytes mixed with variable number of large activated cells. Lymph nodes are diffusely effaced | CD5 CD5 (protein) CD5 is a cluster of differentiation found on a subset of IgM-secreting B cells called B-1 cells, and also on T cells. B-1 cells have limited diversity of their B-cell receptor due to their lack of the enzyme terminal deoxynucleotidyl transferase and are potentially self-reactive... , surface immunoglobulin |
50%. | Occurs in older adults. Usually involves lymph nodes, bone marrow and spleen. Most patients have peripheral blood involvement. Indolent Indolent Indolent may refer to:*Laziness*A music label owned by Bertelsmann Music Group.*the lowest of three grades of non-Hodgkin's lymphoma... . |
| MALT lymphoma 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... |
~5% of lymphomas in adults | Variable cell size and differentiation. 40% show plasma cell Plasma cell Plasma cells, also called plasma B cells, plasmocytes, and effector B cells, are white blood cells which produce large volumes of antibodies. They are transported by the blood plasma and the lymphatic system... differentiation. Homing Homing Homing is the process of determining the location of something, sometimes the source of a transmission, and going to it.More specifically, it may refer to:*Infrared homing*Semi-active radar homing*Active radar homing*Acoustic homing*Homing... of B cells to epithelium creates lymphoepithelial lesions. |
CD5 CD5 (protein) CD5 is a cluster of differentiation found on a subset of IgM-secreting B cells called B-1 cells, and also on T cells. B-1 cells have limited diversity of their B-cell receptor due to their lack of the enzyme terminal deoxynucleotidyl transferase and are potentially self-reactive... , CD10, surface Ig |
Frequently occurs outside lymph nodes. Very indolent. May be cured by local excision. | |
| Burkitt's lymphoma Burkitt's lymphoma Burkitt's lymphoma is a cancer of the lymphatic system... |
< 1% of lymphomas in the United States | Round lymphoid cells of intermediate size with several nucleoli. Starry-sky appearance by diffuse spread with interspersed apoptosis Apoptosis Apoptosis is the process of programmed cell death that may occur in multicellular organisms. Biochemical events lead to characteristic cell changes and death. These changes include blebbing, cell shrinkage, nuclear fragmentation, chromatin condensation, and chromosomal DNA fragmentation... . |
CD10, surface Ig | 50% | Endemic in Africa, sporadic elsewhere. More common in immunocompromised and in children. Often visceral involvement. Highly aggressive. |
| Mycosis fungoides Mycosis fungoides -External links:* * *... |
Most common cutaneous lymphoid malignancy | Usually small lymphoid cells with convoluted nuclei that often infiltrate the epidermis, creating Pautier microabscesses. | CD4 CD4 CD4 is a glycoprotein expressed on the surface of T helper cells, monocytes, macrophages, and dendritic cells. It was discovered in the late 1970s and was originally known as leu-3 and T4 before being named CD4 in 1984... |
75% | Localized or more generalized skin symptoms. Generally indolent. In a more aggressive variant, Sézary's disease Sézary's disease Sézary's disease is a type of cutaneous lymphoma that was first described by Albert Sézary. The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy... , there is skin erythema Erythema Erythema is redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin. It occurs with any skin injury, infection, or inflammation... and peripheral blood involvement. |
| Peripheral T-cell lymphoma-Not-Otherwise-Specified Peripheral T-cell lymphoma-Not-Otherwise-Specified Peripheral T-cell Lymphoma-Not-Otherwise Specified , is a subtype of Peripheral T-cell lymphoma. Peripheral T-cell lymphoma is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells . PTCL is a type of... |
Most common T cell lymphoma | Variable. Usually a mix small to large lymphoid cells with irregular nuclear contours. | CD3 | Probably consists of several rare tumor types. It is often disseminated and generally aggressive. | |
| Nodular sclerosis Nodular sclerosis Nodular sclerosis is a form of Hodgkin's lymphoma that is the most common subtype of HL in developed countries. It affects females and males equally and has a median age of onset at ~28 years... form of Hodgkin lymphoma |
Most common type of Hodgkin's lymphoma | Reed-Sternberg cell Reed-Sternberg cell Reed–Sternberg cells are different giant cells found with light microscopy in biopsies from individuals with Hodgkin's lymphoma primarily due to EBV, and certain other disorders... variants and inflammation. usually broad sclerotic bands that consists of collagen. |
CD15 CD15 CD15 is a cluster of differentiation antigen - an immunologically significant molecule. CD15 is a carbohydrate adhesion molecule that can be expressed on glycoproteins, glycolipids and proteoglycans.- Function :... , CD30 CD30 CD30, also known as TNFRSF8, is a cell membrane protein of the tumor necrosis factor receptor family and tumor marker.This receptor is expressed by activated, but not by resting, T and B cells. TRAF2 and TRAF5 can interact with this receptor, and mediate the signal transduction that leads to the... |
Most common in young adults. It often arises in the mediastinum Mediastinum The mediastinum is a non-delineated group of structures in the thorax, surrounded by loose connective tissue. It is the central compartment of the thoracic cavity... or cervical lymph nodes. |
|
| Mixed-cellularity subtype of Hodgkin lymphoma | Second most common form of Hodgkin's lymphoma | Many classic Reed-Sternberg cells and inflammation | CD15, CD30 | Most common in men. More likely to be diagnosed at advanced stages than the nodular sclerosis form. Epstein-Barr virus Epstein-Barr virus The Epstein–Barr virus , also called human herpesvirus 4 , is a virus of the herpes family and is one of the most common viruses in humans. It is best known as the cause of infectious mononucleosis... involved in 70% of cases. |
See also
- BCP-1 cellsBCP-1 cellsBCP-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 stagingAnn Arbor stagingAnn 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 IndexInternational Prognostic IndexThe International Prognostic Index is a clinical tool developed by oncologists to aid in predicting the prognosis of patients with aggressive non-Hodgkin's lymphoma...
- Epstein barr virus
- LymphadenopathyLymphadenopathyLymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
- Chemotherapy regimensChemotherapy regimensA fundamental philosophy of combination cancer therapy is that different drugs work through different cytotoxic mechanisms. Because they have different dose-limiting adverse effects, they can be given together at full doses in chemotherapy regimens....
External links
- Timeline of discovery and treatment of Hodgkin's Lymphoma
- US lymphoma statistics from the United States National Cancer InstituteNational Cancer InstituteThe National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...
- Hodgkin Lymphoma and UK Non-Hodgkin Lymphoma statistics from the UK
- Latest news and research on Lymphoma
- Lymphoma Imaging Appearance – Chest Radiography
- Lymphoma Association – Specialist UK charity providing free information and support to patients, their families, friends and carers
- Clinically reviewed lymphoma information for patients, from Cancer Research UKCancer Research UKCancer Research UK is a cancer research and awareness charity in the United Kingdom, formed on 4 February 2002 by the merger of The Cancer Research Campaign and the Imperial Cancer Research Fund. Its aim is to reduce the number of deaths from cancer. As the world's largest independent cancer...