Lymphoma

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Lymphoma
Lymphoma macro.jpg
Follicular lymphoma replacing a lymph node
Specialty Hematology and oncology
Symptoms Enlarged lymph nodes, fever, sweats, unintended weight loss, itching, feeling tired [1] [2]
Risk factors Epstein–Barr virus, autoimmune diseases, HIV/AIDS, tobacco smoking [2] [3]
Diagnostic method Lymph node biopsy [1] [2]
Treatment Chemotherapy, radiation therapy, targeted therapy, surgery [1] [2]
PrognosisAverage five year survival 85% (USA) [4]
Frequency4.9 million (2015) [5]
Deaths204,700 (2015) [6]

Lymphoma is a group of blood cancers that develop from lymphocytes (a type of white blood cell). [7] The name often refers to just the cancerous versions rather than all such tumors. [7] Signs and symptoms may include enlarged lymph nodes, fever, drenching sweats, unintended weight loss, itching, and constantly feeling tired. [1] [2] The enlarged lymph nodes are usually painless. [1] The sweats are most common at night. [1] [2]

Lymphocyte Subtype of white blood cell

A lymphocyte is one of the subtypes of a white blood cell in a vertebrate's immune system. Lymphocytes include natural killer cells, T cells, and B cells. They are the main type of cell found in lymph, which prompted the name "lymphocyte".

White blood cell type of cells of the immunological system

White blood cells are the cells of the immune system that are involved in protecting the body against both infectious disease and foreign invaders. All white blood cells are produced and derived from multipotent cells in the bone marrow known as hematopoietic stem cells. Leukocytes are found throughout the body, including the blood and lymphatic system.

Cancer disease of uncontrolled, unregulated and abnormal cell growth

Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal bleeding, prolonged cough, unexplained weight loss, and a change in bowel movements. While these symptoms may indicate cancer, they can also have other causes. Over 100 types of cancers affect humans.

Contents

There are many subtypes of lymphomas. [8] The two main categories of lymphomas are Hodgkin's lymphomas (HL) and the non-Hodgkin lymphomas (NHL). [9] The World Health Organization (WHO) includes two other categories as types of lymphoma: multiple myeloma and immunoproliferative diseases. [10] About 90% of lymphomas are non-Hodgkin lymphomas. [9] [11] Lymphomas and leukemias are a part of the broader group of tumors of the hematopoietic and lymphoid tissues. [12]

Hodgkins lymphoma Lymphoma that is marked by the presence of a type of cell called the Reed-Sternberg cell

Hodgkin's lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cells called lymphocytes. Symptoms may include fever, night sweats, and weight loss. Often there will be non-painful enlarged lymph nodes in the neck, under the arm, or in the groin. Those affected may feel tired or be itchy.

Non-Hodgkin lymphoma cancer of lymph in humans

Non-Hodgkin lymphoma (NHL) is a group of blood cancers that includes all types of lymphoma except Hodgkin's lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss and tiredness. Other symptoms may include bone pain, chest pain or itchiness. Some forms are slow-growing, while others are fast-growing.

World Health Organization Specialised agency of the United Nations

The World Health Organization (WHO) is a specialized agency of the United Nations that is concerned with international public health. It was established on 7 April 1948, and is headquartered in Geneva, Switzerland. The WHO is a member of the United Nations Development Group. Its predecessor, the Health Organisation, was an agency of the League of Nations.

Risk factors for Hodgkin lymphoma include infection with Epstein–Barr virus and a history of the disease in the family. [1] Risk factors for common types of non-Hodgkin lymphomas include autoimmune diseases, HIV/AIDS, infection with human T-lymphotropic virus, immunosuppressant medications, and some pesticides. [2] [13] Eating large amounts of red meat and tobacco smoking may also increase the risk. [3] [14] [15] Diagnosis, if enlarged lymph nodes are present, is usually by lymph node biopsy. [1] [2] Blood, urine, and bone marrow testing may also be useful in the diagnosis. [2] Medical imaging may then be done to determine if and where the cancer has spread. [1] [2] Lymphoma most often spreads to the lungs, liver, and brain. [1] [2]

Epstein–Barr virus Virus of the herpes family

The Epstein–Barr virus (EBV), formally called Human gammaherpesvirus 4, is one of eight known human herpesvirus types in the herpes family, and is one of the most common viruses in humans.

Autoimmune disease Human disease

An autoimmune disease is a condition arising from an abnormal immune response to a normal body part. There are at least 80 types of autoimmune diseases. Nearly any body part can be involved. Common symptoms include low grade fever and feeling tired. Often symptoms come and go.

HIV/AIDS Spectrum of conditions caused by HIV infection

Human immunodeficiency virus infection and acquired immune deficiency syndrome (HIV/AIDS) is a spectrum of conditions caused by infection with the human immunodeficiency virus (HIV). Following initial infection, a person may not notice any symptoms or may experience a brief period of influenza-like illness. Typically, this is followed by a prolonged period with no symptoms. As the infection progresses, it interferes more with the immune system, increasing the risk of developing common infections such as tuberculosis, as well as other opportunistic infections, and tumors that rarely affect people who have uncompromised immune systems. These late symptoms of infection are referred to as acquired immunodeficiency syndrome (AIDS). This stage is often also associated with unintended weight loss.

Treatment may involve one or more of the following: chemotherapy, radiation therapy, targeted therapy, and surgery. [1] [2] In some non-Hodgkin lymphomas, an increased amount of protein produced by the lymphoma cells causes the blood to become so thick that plasmapheresis is performed to remove the protein. [2] Watchful waiting may be appropriate for certain types. [2] The outcome depends on the subtype with some being curable and treatment prolonging survival in most. [9] The five-year survival rate in the United States for all Hodgkin lymphoma subtypes is 85%, [4] while that for non-Hodgkin lymphomas is 69%. [16] Worldwide, lymphomas developed in 566,000 people in 2012 and caused 305,000 deaths. [10] They make up 3–4% of all cancers, making them as a group the seventh-most common form. [10] [17] In children, they are the third-most common cancer. [18] They occur more often in the developed world than the developing world. [10]

Chemotherapy treatment of cancer with one or more cytotoxic anti-neoplastic drugs

Chemotherapy is a type of cancer treatment that uses one or more anti-cancer drugs as part of a standardized chemotherapy regimen. Chemotherapy may be given with a curative intent, or it may aim to prolong life or to reduce symptoms. Chemotherapy is one of the major categories of the medical discipline specifically devoted to pharmacotherapy for cancer, which is called medical oncology.

Radiation therapy therapy using ionizing radiation

Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is therapy using ionizing radiation, generally as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radiation therapy may be curative in a number of types of cancer if they are localized to one area of the body. It may also be used as part of adjuvant therapy, to prevent tumor recurrence after surgery to remove a primary malignant tumor. Radiation therapy is synergistic with chemotherapy, and has been used before, during, and after chemotherapy in susceptible cancers. The subspecialty of oncology concerned with radiotherapy is called radiation oncology.

Targeted therapy

Targeted therapy or molecularly targeted therapy is one of the major modalities of medical treatment (pharmacotherapy) for cancer, others being hormonal therapy and cytotoxic chemotherapy. As a form of molecular medicine, targeted therapy blocks the growth of cancer cells by interfering with specific targeted molecules needed for carcinogenesis and tumor growth, rather than by simply interfering with all rapidly dividing cells. Because most agents for targeted therapy are biopharmaceuticals, the term biologic therapy is sometimes synonymous with targeted therapy when used in the context of cancer therapy. However, the modalities can be combined; antibody-drug conjugates combine biologic and cytotoxic mechanisms into one targeted therapy.

Signs and symptoms

The lymph nodes where lymphoma most commonly develops Diagram showing the lymph nodes lymphoma most commonly develops in CRUK 311.svg
The lymph nodes where lymphoma most commonly develops
Lymphoma and lymphatic system Blausen 0626 lymphoma.png
Lymphoma and lymphatic system

Lymphoma may present with certain nonspecific symptoms; if the symptoms are persistent, an evaluation to determine their cause, including possible lymphoma, should be undertaken.

Lymphadenopathy disorder of lymph nodes

Lymphadenopathy or adenopathy is disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type is lymphadenitis, producing swollen or enlarged lymph nodes. In clinical practice, the distinction between lymphadenopathy and lymphadenitis is rarely made and the words are usually treated as synonymous. Inflammation of the lymphatic vessels is known as lymphangitis. Infectious lymphadenitis affecting lymph nodes in the neck is often called scrofula.

B symptoms refer to systemic symptoms of fever, night sweats, and weight loss which can be associated with both Hodgkin's lymphoma and non-Hodgkin's lymphoma. The presence or absence of B symptoms has prognostic significance and is reflected in the staging of these lymphomas.

Fever of unknown origin (FUO), refers to a condition in which the patient has an elevated temperature (fever) but despite investigations by a physician no explanation has been found.

Diagnosis

Lymphoma may appear as peritoneal lymphomatosis, as can be seen on CT scan. Image depicts non-Hodgkin lymphoma in a 17 year old HIV positive patient. A. Irregular homogenously enhancing wall thickening involving the ileocaecal region with aneurysmal dilatation of involved segments (curved arrow). B. Hepatosplenomegaly with hepatic metastasis (white arrows). CT of abdominal non-Hodgkin lymphoma.jpg
Lymphoma may appear as peritoneal lymphomatosis, as can be seen on CT scan. Image depicts non-Hodgkin lymphoma in a 17 year old HIV positive patient. A. Irregular homogenously enhancing wall thickening involving the ileocaecal region with aneurysmal dilatation of involved segments (curved arrow). B. Hepatosplenomegaly with hepatic metastasis (white arrows).

Lymphoma is definitively diagnosed by a lymph node biopsy, meaning a partial or total excision of a lymph node examined under the microscope. [22] This examination reveals histopathological features that may indicate lymphoma. After lymphoma is diagnosed, a variety of tests may be carried out to look for specific features characteristic of different types of lymphoma. These include:

Classification

Lymph node with mantle cell lymphoma (low-power view, H&E) Hodgkin lymphoma, nodular lymphocyte predominant - low power view - H&E - by Gabriel Caponetti.jpg
Lymph node with mantle cell lymphoma (low-power view, H&E)

Lymphomas in the strict sense are any neoplasms of the lymphatic tissues ( lympho- + -oma ). [23] The main classes are malignant neoplasms (that is, cancers) of the lymphocytes, a type of white blood cell that belongs to both the lymph and the blood and pervades both. Thus, lymphomas and leukemias are both tumors of the hematopoietic and lymphoid tissues, and as lymphoproliferative disorders, lymphomas and lymphoid leukemias are closely related, to the point that some of them are unitary disease entities that can be called by either name (for example adult T-cell leukemia/lymphoma).

Several classification systems have existed for lymphoma, which use histological and other findings to divide lymphoma into different categories. The classification of a lymphoma can affect treatment and prognosis. Classification systems generally classify lymphoma according to:

Lymphoma can also spread to the central nervous system, often around the brain in the meninges, known as lymphomatous meningitis (LM). [24]

Hodgkin lymphoma

Hodgkin lymphoma accounts for about 15% of lymphomas. [25] It differs from other forms of lymphoma in its prognosis and several pathological characteristics. A division into Hodgkin and non-Hodgkin lymphomas is used in several of the older classification systems. A Hodgkin lymphoma is marked by the presence of a type of cell called the Reed–Sternberg cell. [26] [27]

Non-Hodgkin lymphomas

Non-Hodgkin lymphomas, which are defined as being all lymphomas except Hodgkin lymphoma, are more common than Hodgkin lymphoma. A wide variety of lymphomas are in this class, and the causes, the types of cells involved, and the prognosis vary by type. The incidence of non-Hodgkin lymphoma increases with age. It is further divided into several subtypes.

Epstein-Barr virus-associated lymphoproliferative diseases

Epstein-Barr virus-associated lymphoproliferative diseases are a group of benign, pre-malignant, and malignant diseases of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells in which one or more of these cell types is infected with the Epstein-Barr virus (EBV). The virus may be responsible for the development and/or progression of these diseases. In addition to EBV-positive Hodgkin lymphomas, the World Health Organization (2016) includes the following lymphomas, when associated with EBV infection, in this group of diseases: Burkitt lymphoma; large B cell lymphoma, not otherwise specified; diffuse large B cell lymphoma associated with chronic inflammation; fibrin-associated diffuse large cell lymphoma; primary effusion lymphoma; plasmablastic lymphoma; extranodal NK/T cell lymphoma, nasal type; peripheral T cell lymphoma, not otherwise specified; angioimmunoblastic T cell lymphoma; follicular T cell lymphoma; and systemic T cell lymphoma of childhood. [28]

WHO classification

The WHO classification, published in 2001 and updated in 2008, [29] [30] is based upon the foundations laid within the "revised European-American lymphoma classification" (REAL). This system groups lymphomas by cell type (i.e. the normal cell type that most resembles the tumor) and defining phenotypic, molecular, or cytogenetic characteristics. The five groups are shown in the table. Hodgkin lymphoma is considered separately within the WHO and preceding classifications, although it is recognized as being a tumor of, albeit markedly abnormal, lymphocytes of mature B cell lineage.

Of the many forms of lymphoma, some 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). [31]

Lymphoma subtypes (WHO 2008)
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. DNA-microarray analysis.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.
3 to 4% of lymphomas in adults
Small resting lymphocytes mixed with variable numbers of large activated cells, lymph nodes diffusely effaced
CD5, surface immunoglobulin
50%. [32]
Occurs in older adults, usually involves lymph nodes, bone marrow and spleen, most patients have peripheral blood involvement, indolent
About 5% of lymphomas in adults
Variable cell size and differentiation, 40% show plasma cell differentiation, homing of B cells to epithelium creates lymphoepithelial lesions.
CD5, CD10, surface Ig
Frequently occurs outside lymph nodes, very indolent, may be cured by local excision
About 40% of lymphomas in adults
Small "cleaved" cells (centrocytes) mixed with large activated cells (centroblasts), usually nodular ("follicular") growth pattern
CD10, surface Ig
72–77% [33]
Occurs in older adults, usually involves lymph nodes, bone marrow and spleen, associated with t(14;18) translocation overexpressing Bcl-2, indolent
3 to 4% of lymphomas in adults
Lymphocytes of small to intermediate size growing in diffuse pattern
CD5
50% [34] to 70% [34]
Occurs mainly in adult males, usually involves lymph nodes, bone marrow, spleen and GI tract, associated with t(11;14) translocation overexpressing cyclin D1, moderately aggressive
About 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
5-year survival 60% [35]
Occurs in all ages, but most commonly in older adults, may occur outside lymph nodes, aggressive
< 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
CD10, surface Ig
5-year
survival
50% [36]
Endemic in Africa, sporadic elsewhere, more common in immunocompromised and children, often visceral involvement, highly aggressive
Mature T cell and natural killer (NK) cell neoplasms
Most common cutaneous lymphoid malignancy
Usually small lymphoid cells with convoluted nuclei that often infiltrate the epidermis, creating Pautrier microabscesseses
CD4
5-year
survival
75% [37]
Localized or more generalized skin symptoms, generally indolent, in a more aggressive variant, Sézary's disease, skin erythema and peripheral blood involvement
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, often disseminated and generally aggressive
Precursor lymphoid neoplasms
15% of childhood acute lymphoblastic leukemia and 90% of lymphoblastic lymphoma. [29] :635
Lymphoblasts with irregular nuclear contours, condensed chromatin, small nucleoli and scant cytoplasm without granules
TdT, CD2, CD7
It often presents as a mediastinal mass because of involvement of the thymus. It is highly associated with NOTCH1 mutations, and is most common in adolescent males.
Hodgkin lymphoma
Most common type of Hodgkin lymphoma
Reed-Sternberg cell variants and inflammation, usually broad sclerotic bands that consist of collagen
CD15, CD30
Most common in young adults, often arises in the mediastinum or cervical lymph nodes
    • Mixed cellularity Hodgkin lymphoma
Second-most common form of Hodgkin 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 involved in 70% of cases
Immunodeficiency-associated lymphoproliferative disorders

Previous classifications

Several previous classifications have been used, including Rappaport 1956, Lennert / Kiel 1974, BNLI, Working formulation (1982), and REAL (1994).

The Working formulation of 1982 was 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 lymphomas and B-cell lymphomas. It was widely accepted at the time of its publication, but is now obsolete. [38] It is still used by some cancer agencies for compilation of lymphoma statistics and historical rate comparisons. [ citation needed ]

In 1994, the Revised European-American Lymphoma (REAL) classification applied immunophenotypic and genetic features in identifying distinct clinicopathologic entities among all the lymphomas except Hodgkin lymphoma. [39] For coding purposes, the ICD-O (codes 9590–9999) [40] and ICD-10 (codes C81-C96) [41] are available.

Staging

Diagram showing common sites where lymphoma spreads DIagram showing where lymphoma can spread in the body CRUK 382.svg
Diagram showing common sites where lymphoma spreads

After a diagnosis and before treatment, a cancer is staged. This refers to determining if the cancer has spread, and if so, whether locally or to distant sites. Staging is reported as a grade between I (confined) and IV (spread). Staging is carried out because the stage of a cancer impacts its prognosis and treatment. [ citation needed ]

The Ann Arbor staging system is routinely used for staging of both HL and NHL. In this staging system, I represents a localised disease contained within a lymph node group, II represents the presence of lymphoma in two or more lymph nodes groups, III represents spread of the lymphoma to lymph nodes groups on both sides of the diaphragm, and IV indicates spread to tissue outside the lymphatic system. Different suffixes imply involvement of different organs, for example S for the spleen and H for the liver. Extra-lymphatic involvement is expressed with the letter E. In addition, the presence of B symptoms or their absence is expressed with B and A, respectively. B symptoms are defined as the presence of one of three symptoms: Unintentional weightloss of 10% body weight in the last 6 months, night sweats, and persistent fever of 38 °C or more. [42]

CT scan or PET scan imaging modalities are used to stage a cancer. PET scan is advised for FDG avid lymphomas, for example Hodgkins Lymphoma as a staging tool that can even replace bone marrow biopsy. For other lymphomas CT scan is recommended for staging. [43]

Age and poor performance status are established poor prognostic factors, as well. [44]

Differential diagnosis

Certain lymphomas (extranodal NK/T-cell lymphoma, nasal type and type II enteropathy-associated T-cell lymphoma) can be mimicked by two benign diseases which involve the excessive proliferation of non-malignant NK cells in the GI tract, natural killer cell enteropathy, a disease wherein NK cell infiltrative lesions occur in the intestine, colon, stomach, or esophagus, and lymphomatoid gastropathy, a disease wherein these cells' infiltrative lesions are limited to the stomach. These diseases do not progress to cancer, may regress spontaneously and do not respond to, and do not require, chemotherapy or other lymphoma treatments. [45]

Treatment

Prognoses and treatments are different for HL and between all the different forms of NHL, [46] and also depend on the grade of tumour, referring to how quickly a cancer replicates. Paradoxically, high-grade lymphomas are more readily treated and have better prognoses: [ citation needed ] Burkitt lymphoma, for example, is a high-grade tumour known to double within days, and is highly responsive to treatment. Lymphomas may be curable if detected in early stages with modern treatment.

Low-grade

Many low-grade lymphomas remain indolent for many years. Treatment of the nonsymptomatic patient is often avoided. In these forms of lymphoma, such as follicular lymphoma, watchful waiting is often the initial course of action. This is carried out because the harms and risks of treatment outweigh the benefits. [47] 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. Some centers advocate the use of single agent rituximab in the treatment of follicular lymphoma rather than the wait and watch approach. Watchful waiting is not a good strategy for all patients, as it leads to significant distress and anxiety in some patients. It has been equated with watch and worry. [48]

High-grade

Treatment of some other, more aggressive, forms of lymphoma [ which? ] can result in a cure in the majority of cases, but the prognosis for patients with a poor response to therapy is worse. [49] Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP or R-CHOP regimen. A number of people are cured with first-line chemotherapy. Most relapses occur within the first two years, and the relapse risk drops significantly thereafter. [50] For people who relapse, high-dose chemotherapy followed by autologous stem cell transplantation is a proven approach. [51]

Hodgkin lymphoma

Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized. [52]

Advanced Hodgkin disease requires systemic chemotherapy, sometimes combined with radiotherapy. [53] Chemotherapy used includes the ABVD regimen, which is commonly used in the United States. Other regimens used in the management of Hodgkin lymphoma include BEACOPP and Stanford V. Considerable controversy exists regarding the use of ABVD or BEACOPP. Briefly, both regimens are effective, but BEACOPP is associated with more toxicity. Encouragingly, a significant number of people who relapse after ABVD can still be salvaged by stem cell transplant. [54]

Palliative care

Palliative care, a specialized medical care focused on the symptoms, pain, and stress of a serious illness, is recommended by multiple national cancer treatment guidelines as an accompaniment to curative treatments for people suffering from lymphoma. [55] [56] It is used to address both the direct symptoms of lymphoma and many unwanted side effects that arise from treatments. [57] [58] Palliative care can be especially helpful for children who develop lymphoma, helping both children and their families deal with the physical and emotional symptoms of the disease. [57] [59] [60] [61] For these reasons, palliative care is especially important for patients requiring bone marrow transplants. [62] [63]

Prognosis

Five-year relative survival by stage at diagnosis [64]
Stage at diagnosisFive-year relative
survival (%)
Percentage
of cases (%)
Localized (confined to primary site)82.326
Regional (spread to regional lymph nodes)78.319
Distant (cancer has metastasized)62.747
Unknown (unstaged)68.68

Epidemiology

Deaths from lymphomas and multiple myeloma per million persons in 2012
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0-13
14-18
19-22
23-28
29-34
35-42
43-57
58-88
89-121
122-184 Lymphomas, multiple myeloma world map-Deaths per million persons-WHO2012.svg
Deaths from lymphomas and multiple myeloma per million persons in 2012
  0-13
  14-18
  19-22
  23-28
  29-34
  35-42
  43-57
  58-88
  89-121
  122-184

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. [65]

According to the U.S. National Institutes of Health, lymphomas account for about 5%, and Hodgkin lymphoma in particular accounts for less than 1% 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. [66]

History

Thomas Hodgkin Thomas Hodgkin photo.jpg
Thomas Hodgkin

Thomas Hodgkin published the first description of lymphoma in 1832, specifically of the form named after him. [67] Since then, many other forms of lymphoma have been described.

Research

The two types of lymphoma research are clinical or translational research and basic research. Clinical/translational research focuses on studying the disease in a defined and generally immediately patient-applicable way, such as testing a new drug in patients. Studies may focus on effective means of treatment, better ways of treating the disease, improving the quality of life for patients, or appropriate care in remission or after cures. Hundreds of clinical trials are being planned or conducted at any given time. [68]

Basic science research studies the disease process at a distance, such as seeing whether a suspected carcinogen can cause healthy cells to turn into lymphoma cells in the laboratory or how the DNA changes inside lymphoma cells as the disease progresses. The results from basic research studies are generally less immediately useful to patients with the disease, [69] but can improve scientists' understanding of lymphoma and form the foundation for future, more effective treatments.

Other animals

Related Research Articles

Chronic lymphocytic leukemia lymphoblastic leukemia characterized by over production of B-cells and their accumulation in bone marrow and blood

Chronic lymphocytic leukemia (CLL) is a type of cancer in which the bone marrow makes too many lymphocytes. Early on there are typically no symptoms. Later non-painful lymph nodes swelling, feeling tired, fever, or weight loss for no clear reason may occur. Enlargement of the spleen and a low red blood cells (anemia) may also occur. It typically worsens gradually.

Tumors of the hematopoietic and lymphoid tissues tumor that affect the blood, bone marrow, lymph, and lymphatic system

Tumors of the hematopoietic and lymphoid tissues or tumours of the haematopoietic and lymphoid malignancies are tumors that affect the blood, bone marrow, lymph, and lymphatic system. As those elements are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making myeloproliferation and lymphoproliferation closely related and often overlapping problems.

Acute lymphoblastic leukemia leukemia that is characterized by over production of lymphoblasts.

Acute lymphoblastic leukemia (ALL) is a cancer of the lymphoid line of blood cells characterized by the development of large numbers of immature lymphocytes. Symptoms may include feeling tired, pale skin color, fever, easy bleeding or bruising, enlarged lymph nodes, or bone pain. As an acute leukemia, ALL progresses rapidly and is typically fatal within weeks or months if left untreated.

Anaplastic large-cell lymphoma non-Hodgkin lymphoma involving aberrant T-cells

Anaplastic large-cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma involving aberrant T cells or null lymphocytes. It is described in detail in the "Classification of Tumours of the Haematopoietic and Lymphoid Tissues" edited by experts of the World Health Organisation (WHO). The term anaplastic large-cell lymphoma (ALCL) encompasses at least four different clinical entities, all sharing the same name, which histologically share the presence of large pleomorphic cells that express CD30 and T-cell markers. Two types of ALCL present as systemic disease and are considered as aggressive lymphomas, while two types present as localized disease and may progress locally. Anaplastic large cell lymphoma is associated with various types of medical implants.

Lymphoid leukemias are a group of leukemias affecting circulating lymphocytes, a type of white blood cells. The lymphocytic leukemias are closely related to lymphomas of the lymphocytes, to the point that some of them are unitary disease entities that can be called by either name. Such diseases are all lymphoproliferative disorders. Most lymphoid leukemias involve a particular subtype of lymphocytes, the B cells.

Sézary disease type of cutaneous lymphoma

Sézary 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 disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy. There are currently no known causes of Sézary disease.

Lymphoma in animals

Lymphoma (lymposarcoma) in animals is a type of cancer defined by a proliferation of malignant lymphocytes within solid organs such as the lymph nodes, bone marrow, liver and spleen. The disease also may occur in the eye, skin, and gastrointestinal tract.

B-cell lymphoma non-Hodgkin lymphoma that has material basis in B cells

The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. They develop more frequently in older adults and in immunocompromised individuals.

Splenic marginal zone lymphoma (SMZL) is a type of cancer 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 lymphoblasts, and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen. Frequently, the bone marrow and splenic hilar lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villous lymphocytes due to their characteristic appearance.

T-cell prolymphocytic leukemia A slow-growing type of leukemia (blood cancer) in which too many lymphocytes are found in the bone marrow and/or blood. The T-cell is specified as the defective cell line.

T-cell-prolymphocytic leukemia (T-PLL) 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 leukemias in adults. Other names include T-cell chronic lymphocytic leukemia, "knobby" type of T-cell leukemia, and T-prolymphocytic leukemia/T-cell lymphocytic leukemia.

Aggressive NK-cell leukemia Human disease

Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells and a rapidly declining clinical course.

Angioimmunoblastic T-cell lymphoma is a mature T-cell lymphoma of blood or lymph vessel immunoblasts characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs) and systemic involvement.

Mantle cell lymphoma B-cell lymphocytic neoplasm due to CD5 positive antigen-naive pregerminal center B-cell within the mantle zone that surrounds normal germinal center follicles

Mantle cell lymphoma (MCL) is a type of non-Hodgkin's lymphoma (NHL), comprising about 6% of NHL cases. There are only about 15,000 patients presently in the U.S.

Marginal zone B-cell lymphoma B-cell lymphoma that is characterized by initial formation in the marginal zones of lymph tissue

Marginal Zone B-cell Non-Hodgkins Lymphoma (NHL) is a type of lymphoma that affects B-cells in the marginal zones of various areas. Marginal zone lymphomas are slow growing and make up about 12% of all B-cell NHL. The median age for diagnosis is 65 years old and is primarily present in the stomach, intestines, salivary glands, lung, thyroid gland, lacrimal gland, conjunctiva, bladder, kidney, skin, soft tissue, thymus gland, and breast. The three types of marginal zone lymphomas include extranodal marginal zone lymphoma (MALT), nodal marginal zone lymphoma, and splenic marginal zone lymphoma. Marginal zone lymphomas are those that develop in the marginal zone or edge of the lymphoid tissue where B-cells are located. All marginal zone lymphomas are low-grade B-cell NHL. Nodal MZL makes up less than 2 in 100 NHL cases, MALT lymphoma makes up 1 in 13 NHL cases, and splenic marginal zone NHL makes up less than 2 in 100 NHL cases. Symptoms tend to vary from each individual patient and are often not enough to make an immediate diagnosis, as this cancer may have similar symptoms to other diseases. Prognosis and treatment are dependent on the location of the cancer and the stage of diagnosis.

Nodular lymphocyte predominant Hodgkins lymphoma

Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is an indolent CD20(+) form of lymphoma.

Lutzner cells

Lutzner cells were discovered by Marvin A. Lutzner, Lucien-Marie Pautrier, and Albert Sézary. These cells are also referred to as Pautrier’s abscess, Sézary’s cell, or Sézary-Lutzner cells. They are a form of T-lymphocytes that has been mutated This atypical form of T-lymphocytes contains T-cell receptors on the surface and is found in both the dermis and epidermis layers of the skin. Since Lutzner cells are a mutated form of T-lymphocytes, they develop in bone marrow and are transported to the thymus is order to mature. The production and maturation stages occur before the cell has developed a mutation. Lutzner cells can form cutaneous T-cell lymphoma, which is a form of skin cancer.

Epstein–Barr virus-associated lymphoproliferative diseases are a group of disorders in which one or more types of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells, are infected with the Epstein–Barr virus (EBV), proliferate excessively, and are associated with the development of various non-malignant, pre-malignant, and malignant lymphoproliferative disorders (LPD). These LPD include the well-known disorder occurring during the initial infection with the EBV, infectious mononucleosis, and the large number of subsequent disorders that may occur thereafter. The virus is usually involved in the development and/or progression of these LPD although in some cases it may be an "innocent" bystander, i.e. present in, but not contributing to, the disease.

Indolent T cell lymphoproliferative disorder of the gastrointestinal tract or Indolent T cell lymphoproliferative disorder of the GI tract (ITCLD-GT) is a rare and recently recognized disorder in which mature T cell lymphocytes accumulation abnormally in the gastrointestinal tract. This accumulation causes various lesions in the mucosal layer lining the GI tract. Individuals with ITCLD-GT commonly complain of chronic GI tract symptoms such as nausea, vomiting, diarrhea, abdominal pain, and rectal bleeding.

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