Lymphoma Subtypes

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There are more than 60 subtypes of lymphoma. Lymphoma Coalition has listed the lymphoma subtypes by Hodgkin, B-cell, T-cell, and CLL.

B-cell and T-cell lymphomas were formerly classified as NHL (non-hodgkin lymphomas).

 

Hodgkin Lymphomas

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Classical Hodgkin lymphoma - Nodular sclerosis:

Nodular Sclerosis CHL is the most common subtype of HL, accounting for 60 to 80 percent of all HL cases. In nodular (knot-like) sclerosis CHL, the involved lymph nodes contain RS cells mixed with normal white blood cells. The lymph nodes often contain a lot of scar tissue, which is where the name nodular sclerosis (scarring) originates. The disease is more common in women than in men, and it usually affects adolescents and adults under the age of 50. The majority of patients are cured with current treatments.

 

Classical Hodgkin lymphoma - Lymphocyte-rich:

Lymphocyte-Rich CHL accounts for less than five percent of HL cases. The disease may be diffuse (spread out) or nodular in form and is characterized by the presence of numerous normal-appearing lymphocytes and classic RS cells. This subtype of HL is usually diagnosed at an early stage in adults and has a low relapse (disease returns after treatment) rate.

 

Classical Hodgkin lymphoma - Mixed cellularity:

Mixed Cellularity CHL accounts for about 15 to 30 percent of all HL cases. The disease is found more commonly in men than in women, and it primarily affects older adults. With this type of CHL, the lymph nodes contain many RS cells in addition to several other cell types. More advanced disease is usually present by the time this subtype is diagnosed.

 

Classical Hodgkin lymphoma - Lymphocyte-depleted:

Lymphocyte-Depleted CHL is rarely diagnosed. Abundant Reed Sternberg (RS) cells and few normal lymphocytes are present in the lymph nodes of patients with this subtype, which is aggressive and usually not diagnosed until it is widespread throughout the body.

 

Classical Hodgkin lymphoma

(Not otherwise specified)

 

Nodular lymphocyte predominant Hodgkin lymphoma:

About 1 in 20 Hodgkin lymphomas diagnosed (5%) are NLPHL. It tends to grow more slowly and to be diagnosed earlier, when it is only growing in one or two groups of lymph nodes. The lymph nodes it grows in tend to be in places that can be easily felt, rather than deep inside the body. For these reasons, treatment is usually very successful and many people are cured.

 

B-Cell Lymphomas 

(formerly classified as NHL)

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Precursor B-cell:

A precursor B-cell, called a B-cell lymphoblast, is an immature lymphocyte that is eventually destined to become a mature B-cell. It is this cell that becomes cancerous in precursor B-cell lymphoblastic lymphoma.

Precursor B-cell lymphoblastic lymphoma is a type of aggressive lymphoma that occurs mainly in children and adolescents, with two-thirds being male. A second peak of occurrence happens later in life in people over 40 years of age.

 

B-cell lymphoma

(Not otherwise specified)

 

Burkitt's lymphoma:

Burkitt's lymphoma is a type of high-grade lymphoma that develops from B lymphocytes (B cells). It is one of the fastest growing lymphomas. This means though that it is more likely to be cured by treatment than many other types of lymphoma.

Burkitt's lymphoma is named after a doctor called Denis Burkitt. He first wrote about a form of this lymphoma after seeing children and young adults with it in Africa. The kind of Burkitt's lymphoma he described is now called ‘African-type’ (or ‘endemic’) Burkitt lymphoma. Another kind (the ‘sporadic’ type) is more often seen in the Western world.

 

Cutaneous B-cell lymphoma:

Primary cutaneous B-cell lymphomas (CBCLs) occur when the lymphoma cells originate in B-cells. CBCLs are nearly always indolent (slow-growing). They may appear on the skin as a reddish rash, lump, or nodule and may have a slightly raised and smooth appearance. The disease tends to go away and then return on the skin, but it rarely develops into a disease that affects other areas of the body. Nearly 50 percent of patients diagnosed with CBCL experience a recurrence after an initial complete response to treatment. Prognosis is usually very good.

 

Diffuse large B-cell lymphoma

(Not otherwise specified)

 

DLBCL - Activated B-Cell (ABC)

Activated B-cell (ABC) DLBCL is derived from B cells that are in the process of differentiating from germinal center B cells to plasma cells. Treatment for ABC type DLBCL varies from GCB type, making it vital that the cell of origin is known prior to treatment.

 

DLBCL - Germinal Center B-Cell (GCB)

Germinal center B-cell (GCB) DLBCL has a gene expression profile that is characteristic of B cells of the germinal center of the lymph node.

 

DLBCL - Intravascular large B-cell lymphoma:

Intravascular large B-cell lymphoma affects small blood vessels. With this B-cell type of lymphoma, cancer cells circulate in the blood and block (occlude) small blood vessels. Intravascular large B-cell lymphoma may also be called intravascular lymphomatosis or malignant angioendotheliomatosis.

 

DLBCL - Mediastinal large B-cell lymphoma:

Primary mediastinal large B-cell lymphoma (PMBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL). It occurs in the thymus glandthymus gland, the small gland in the centre of the chest behind the breastbone (sternum) where lymphocytes (a type of white blood cell that fights germs, foreign substances or cancer cells) mature, multiply and become T cells. Or it occurs in lymph nodes in the centre of the chest (mediastinum), the space in the chest between the lungs, breastbone and spine that contains the heart, great blood vessels, thymus, trachea (windpipe), esophagus and lymph nodes.

This B-cell type of lymphoma is not common. It accounts for only a small number of all lymphomas. Primary mediastinal large B-cell lymphoma may also be called mediastinal B-cell lymphoma or mediastinal (thymic) large B-cell lymphoma.

 

DLBCL - Primary effusion lymphoma:

Primary effusion lymphoma (PEL) is a rare HIV-associated lymphoma that accounts for approximately 4% of all HIV-associated lymphomas. PEL has a unique clinical presentation in having a predilection for arising in body cavities such as the pleural space, pericardium, and peritoneum.

 

Extranodal Marginal-zone lymphoma (MALT):

Extranodal marginal zone lymphoma or mucosa-associated lymphoid tissue (MALT) is the most common form of marginal zone lymphoma. It occurs outside the lymph nodes, in places such as the stomach, small intestine, salivary gland, thyroid, eyes, and lungs. MALT lymphoma is divided into two categories: gastric, which develops in the stomach, and non-gastric, which develops outside of the stomach. This form of lymphoma makes up about nine percent of all B-cell lymphomas.

 

Follicular lymphoma:

Follicular lymphoma (FL), a B-cell lymphoma, is the most common indolent (slow-growing) form of lymphoma, accounting for approximately 20 percent to 30 percent of all NHLs. Common signs of disease include enlargement of the lymph nodes in the neck, underarm, stomach, or groin, as well as fatigue, shortness of breath, night sweats, and weight loss. Often, people with FL have no obvious symptoms of the disease at diagnosis.

 

Hairy-cell leukaemia:

Hairy cell leukaemia (HCL) is a type of low-grade lymphoma. It develops from blood cells called lymphocytes. HCL gets its unusual name because the surface of the cancerous lymphocytes looks ruffled and hairy under the microscope. It is called a ‘leukaemia’ because the lymphocytes can be found in the blood, though they mainly collect in the bone marrow and the spleen. Less commonly, they collect in the liver or in lymph nodes, for example in lymph nodes in the abdomen.

 

Heavy chain disease:

Heavy chain diseases (HCD) include three rare B-cell neoplasms that produce monoclonal heavy chains and typically no light chains. The monoclonal immunoglobulin component is composed of either IgG (Gamma HCD), IgA (Alpha HCD), or IgM (Mu HCD).

 

Mantle-cell lymphoma:

Mantle cell lymphoma (MCL) is a rare, B-cell NHL that most often affects men over the age of 60. The disease may be aggressive (fast growing) but it can also behave in a more indolent (slow growing) fashion in some patients. MCL comprises about five percent of all NHLs. The disease is called "mantle cell lymphoma" because the tumor cells originally come from the "mantle zone" of the lymph node. MCL is usually diagnosed as a late-stage disease that has typically spread to the gastrointestinal tract and bone marrow.

 

Nodal Marginal-zone lymphoma:

Nodal marginal zone lymphoma (NMZL) is often referred to as ‘monocytoid B-cell lymphoma’ and is usually slow-growing. Whilst it is unlikely, NMZL does have the ability to transform into a more aggressive type of lymphoma.

 

Primary Central Nervous System (CNS) Lymphoma:

Primary central nervous system (CNS) lymphoma (PCNSL) is a type of lymphoma that starts in the brain or spinal cord. 

Splenic Marginal-zone lymphoma:

Splenic MZL is a low-grade B-cell lymphoma that develops in the spleen. The lymphoma is usually also present in the bone marrow and is often found in the blood too. It can affect the internal lymph nodes next to the spleen and it can affect the liver. 

 

Waldenstrom macroglubulinemia/Lymphoplasmacytic lymphoma:

WM occurs in a type of white blood cell called a B-lymphocyte or B-cell, which normally matures into a plasma cell whose job is to manufacture immunoglobulins (antibodies) to help the body fight infection. In WM, there is a malignant change to the B-cell in the late stages of maturing, and it continues to proliferate into a clone of identical cells, primarily in the bone marrow but also in the lymph nodes and other tissues and organs of the lymphatic system. These clonal cells over-produce an antibody of a specific class called IgM.

 

T-Cell Lymphomas

(formerly classified as NHL)

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Precursor T-Cell:

A precursor T-cell, called a T-cell lymphoblast, is an immature lymphocyte that is eventually destined to become a mature T-cell. It is this cell that becomes cancerous in precursor T-cell lymphoblastic lymphoma. Precursor T-cell lymphoblastic lymphoma is a type of aggressive lymphoma that occurs mainly in children and adolescents, and more often in males than females. A second peak of occurrence is seen later in life in people over 40 years of age.

 

Adult T-cell leukaemia/lymphoma:

Anaplastic large-cell lymphoma (ALCL) is a rare type of aggressive T-cell lymphoma comprising about 3 percent of all lymphomas in adults and between 10 percent and 30 percent of all lymphomas in children. It can present either systemically (meaning in organs throughout the body) or cutaneously (on the surface of the skin). Systemic anaplastic large-cell lymphoma may respond well to chemotherapy treatment and is potentially curable.

When ALCL is confined to the skin, it follows a less aggressive course and is associated with a rare condition called lymphomatoid papulosis (LyP), which, though not classified as a lymphoma, is often a precursor to development of cutaneous anaplastic large-cell lymphoma. Patients with systemic ALCL are divided into two groups, depending on the expression of a protein called anaplastic lymphoma kinase (ALK). The prognosis for ALCL depends on whether a patient is ALK positive (expresses the protein) or ALK negative (does not express the protein). ALK positive disease responds well to chemotherapy, putting most patients in long-term remission or cure. A majority of ALK negative patients will relapse within five years and are treated more aggressively, often with transplant.

 

Anaplastic large cell lymphoma:

ALCL comprises approximately 20 percent of all T-cell lymphomas. Initial symptoms of ALCL can include fever, backache, painless swelling of lymph nodes, loss of appetite, and tiredness.

ALCL can initially appear either in the skin, in lymph nodes, or in organs throughout the body. ALCL that appears in the skin is called primary cutaneous ALCL, which has a less aggressive disease course than the systemic types. 

 

Angioimmunoblastic T-cell lymphoma:

Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive (fast-growing) form of peripheral T-cell lymphoma (PTCL). AITL is one of the more common subtypes of mature T-cell lymphomas. Symptoms of AITL include high fever, night sweats, skin rash, and autoimmune disorders such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). As a result of these autoimmune disorders, the body’s immune system does not recognize, and consequently destroys, its own cells and tissues, such as red blood cells (in the case of AIHA) or platelets (in the case of ITP).

 

Composite lymphoma:

A rare form of lymphoma in which different types of lymphoma cells occur at the same time. 

 

Cutaneous T-cell lymphoma:

(includes mycosis fungoides and sezary syndrome)

Cutaneous T-cell lymphoma (CTCL) is the most common type of cutaneous lymphoma that typically presents with red, scaly patches or thickened plaques of skin that often mimic eczema or chronic dermatitis. Progression from limited skin involvement is variable and may be accompanied by tumor formation, ulceration, and exfoliation, complicated by itching and infections. Advanced stages are defined by involvement of lymph nodes, peripheral blood, and internal organs. Most CTCLs typically fall into the category of indolent (i.e. chronic) lymphomas – treatable, but not curable and usually not life-threatening.

 

Enteropathy-type T-cell lymphoma:

Enteropathy-type T-cell lymphoma is an extremely rare subtype of T-cell lymphoma that appears in the intestines and is strongly associated with celiac disease.

 

Hepatosplenic T-cell lymphoma:

Hepatosplenic gamma-delta T-cell lymphoma is an extremely rare and aggressive disease that starts in the liver or spleen. This lymphoma may occur in people with Crohn’s disease whose immune system is suppressed. As with other rare cancers, patients should discuss treatment options with their medical team.

 

Lymphoid neoplasm

(Not otherwise specified)

 

NK/T-cell lymphoma:

It is a fast-growing lymphoma that typically originates in the lining of the nose or upper airway. It is treated with radiation and various combinations of chemotherapy.

 

Peripheral T-cell lymphoma:

One of a group of aggressive lymphomas that begin in mature T lymphocytes (T cells that have matured in the thymus gland and gone to other lymphatic sites in the body, including lymph nodes, bone marrow, and spleen.) Also called mature T-cell lymphoma.

 

Precursor lymphoblastic leukaemia/lymphoma:

(unknown lineage)

An aggressive type of leukaemia in which too many T-cell lymphoblasts  are found in the bone marrow and blood.

 

Primary cutaneous anaplastic large cell lymphoma:

ALCL comprises approximately 20 percent of all T-cell lymphomas. Initial symptoms of ALCL can include fever, backache, painless swelling of lymph nodes, loss of appetite, and tiredness.

ALCL can initially appear either in the skin, in lymph nodes, or in organs throughout the body. ALCL that appears in the skin is called primary cutaneous ALCL, which has a less aggressive disease course than the systemic types. The characteristic features of primary cutaneous ALCL include the appearance of solitary or multiple raised red skin lesions that do not go away, have a tendency to ulcerate, and may itch. These ALCL lesions are tumors, and they can appear on any part of the body, often grow very slowly, and may be present for a long time before being diagnosed. Approximately 10 percent of the time, primary cutaneous ALCL extends beyond the skin to lymph nodes or organs. If this occurs, it is usually treated like the systemic forms of ALCL.

 

Prolymphocytic leukaemia:

(unknown lineage)

A type of chronic lymphocytic leukaemia (CLL), in which too many immature white blood cells (prolymphocytes) are found in the blood and bone marrow. Prolymphocytic leukaemia usually progresses more rapidly than classic CLL. Also called PLL.

 

Subcutaneous panniculitis-like T-cell lymphoma:

This is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk.

 

T-cell large granular lymphocytic leukaemia:

T-cell large granular lymphocytic (TLGL) leukaemia is a rare lymphoproliferative cancer of T-cell origin. Lymphoproliferative means that T lymphocytes are produced in large amounts. TLGL leukaemia is characterized by large granular lymphocytes, which are bigger than normal lymphocytes. It may also be called T-cell granular lymphocytic leukaemia.

TLGL leukaemia occurs most often in older adults. The average age at diagnosis is about 60. However, it can occur in all age groups.

 

T-Cell lymphoma

(Not otherwise specified, unknown lineage)

 

T-cell Non-Hodgkin lymphoma

(Not otherwise specified)

 

T-cell Prolymphocytic leukaemia:

Prolymphocytic leukaemias (PLLs) are rare lymphoid leukaemias, which account for only about 2% of all mature lymphoid leukaemias. PLLs present like leukaemia and, like lymphomas, they start in the lymphocytes, but do not form solid tumours. PLLs are also considered lymphoproliferative disorders, which mean that lymphocytes are produced in large amounts. PLLs were thought to be a rare variation of chronic lymphocytic leukaemia (CLL), but are now considered a distinct disease.

 

CLL

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Chronic/Small lymphocytic leukaemia/lymphoma:

CLL and SLL are essentially the same disease, the only difference being where the cancer primarily occurs. When most of the cancer cells are located in the bloodstream and the bone marrow, the disease is referred to as CLL, although the lymph nodes and spleen are often involved. When the cancer cells are located mostly in the lymph nodes, the disease is called SLL.


Many patients with CLL/SLL do not have any obvious symptoms of the disease. Their doctors might detect the disease during routine blood tests and/or a physical examination. For others, the disease is detected when symptoms occur and the patient goes to the doctor because he or she is worried, uncomfortable, or does not feel well. CLL/SLL may cause different symptoms depending on the location of the tumor in the body.

The symptoms of CLL/SLL include a tender, swollen abdomen and feeling full even after eating only a small amount. Other symptoms of CLL/SLL can include fatigue, shortness of breath, anemia, bruising easily, night sweats, weight loss, and frequent infections. However, many patients with CLL/SLL will live for years without symptoms.

 

Prolymphocytic B-cell leukaemia:

A type of chronic lymphocytic leukaemia (CLL), in which too many immature white blood cells (prolymphocytes) are found in the blood and bone marrow. Prolymphocytic leukaemia usually progresses more rapidly than classic CLL. Also called PLL.

 

 

 

Sources:

Lymphoma Research Foundation (www.lymphoma.org)
Accessed March 2016

National Institute of Cancer, (http://seer.cancer.gov/lymphomarecode/lymphoma-who2008.html), Accessed March 2016

Lymphoma Canada, (www.lymphoma.ca), Accessed March 2016

Lymphoma Australia, (https://www.lymphoma.org.au), Accessed March 2016

International Waldenstrom's Macroglobulinemia Foundation (IWMF), (http://www.iwmf.com/about-wm/what-wm), Accessed March 2016

 

 

 

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