Non-Hodgkin's LymphomaTypes |
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Types
Throughout the past 40 years, the classification of lymphoma has changed considerably. These changes have been based upon new insights provided by technological advances, as well as advances in our understanding of the clinical behavior of lymphoma.
Like Hodgkin's disease, non-Hodgkin's lymphoma is composed of a very mixed group of cancers. There are many subtypes, each with a different prognosis. The loosely divided classifications, as recognized by the International Lymphoma Study Group (ILSG), are:
- Indolent (
- Moderately aggressive (intermediate grade) lymphomas and lymphoid leukemias
- Aggressive (high grade) lymphomas
- Highly aggressive lymphomas and lymphoid leukemias
NHLs may be separated further according to cell type: B-cell tumors are composed of B-cell variants, while T-cell tumors are composed of T cell. Among NHLs, roughly 85% of cases are B-cell cancers, whereas only 15% are T-cell cancers.
In the normal immune system, B-cells (B-lymphocytes) fight bacteria by developing into plasma cells. The plasma cells make antibodies that "tag" germs for removal by specialized immunologic cells which surround and digest them. By contrast, T-cells (T lymphocytes) are immunologic "conductors." They direct B-cells and other lymphatic cells in the production of an overall immune response. T-cells help to rid the body of viruses, fungi, and certain bacteria, and they also make cytokines - substances that draw other infection-fighting white blood cells to tissues that are under attack by microorganisms.
Table 2: NHL Groups
| Suggested NHL groups recognized by the ILSG: | ||
| I. Slow-Growing Lymphomas and Lymphoid Leukemias | ||
| B-cell |
B-Cell Chronic Lymphocytic Leukemia (B-CLL)/Small Lymphocytic Lymphoma (SLL) |
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| T-cell |
Large Granular Lymphocyte Leukemia Adult T-Cell Leukemia/Lymphoma (ATL/L ) [smoldering] Mycosis Fungoides/Sézary Syndrome |
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| II. Moderately Aggressive Lymphomas and Lymphoid Leukemias | |
| B-cell |
B-Cell Prolymphocytic Leukemia (B-PLL) Mantle Cell Lymphoma Follicle Center Lymphoma, Follicular Small Cleaved Cell (FSC), Follicle Center Lymphoma (follicular large cell)
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| T-cell |
T-cell Chronic Lymphocytic Leukemia/Prolymphocytic Leukemia (T-CLL/PLL) Adult T-Cell Leukemia/Lymphoma (ATL/L) [chronic] Angiocentric Lymphoma Angioimmunoblastic Lymphoma |
| III. Aggressive Lymphomas | ||
| B-cell |
B-Cell Large B-Cell Lymphoma
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| T-cell |
Peripheral T-Cell Lymphomas Intestinal T-Cell Lymphoma Anaplastic Large Cell Lymphoma
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| IV. Highly Aggressive Lymphomas and Lymphoid Leukemias | |
| B-cell |
Precursor B-Lymphoblastic Leukemia/Lymphoma (PB-LBL/L) Burkitt's Lymphoma High-Grade B-Cell Lymphoma, Burkitt's-like
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| T-cell |
Precursor T-lymphoblastic leukemia/lymphoma (T-LBL/L) Adult T-cell leukemia/Lymphoma (ATLL) [acute and lymphomatous] |
Slow-Growing (Low Grade) Lymphomas
Slow-growing lymphomas are subdivided into numerous B-cell and T-cell subtypes, for example:
| B-cell |
Small lymphocytic / pro-lymphocytic lymphoma (SLL) |
| T-cell |
Large granular lymphocyte leukemia Adult T-cell leukemia/lymphoma (ATL/L ) Mycosis fungoides/Sézary syndrome |
Small lymphocytic/prolymphocytic lymphoma (B-SLL) represents only about 4% of adult NHL. It usually occurs in adults with generalized lymph node disease (lymphadenopathy). This disease manifests more often as B-cell chronic lymphocytic leukemia (B-CLL) than as B-SLL. An increased rate of cell division is associated with more aggressive disease and a shorter survival time. The lymph node cells of SLL may transform into an aggressive, large B-cell lymphoma in as many as 15% of patients. This is known as Richter's syndrome. In addition, B-CLL/SLL may undergo a Hodgkin's-like transformation.
Follicular lymphoma represents 70% of all the low-grade B-cell lymphomas. It is the second most common lymphoma in the United States and comprises 20% of all NHL. Men and women are affected in nearly equal numbers, and the average age of onset is 55 years. Most patients tend to have widespread, advanced-stage disease at diagnosis. Despite this fact, the disease is slow to progress, although tumor grade may play a role in disease behavior. Only one-quarter of affected individuals develop B symptoms. The bone marrow becomes a site of disease spread in nearly half of all patients. Transformation to an aggressive lymphoma is a frequent, deadly consequence of this disease. In such cases, the follicular pattern changes from a low percentage of large cells to a more diffuse pattern with primarily large cells.
In many patients, lymphoplasmacytoid lymphoma conforms to the clinical picture of Waldenstrom's macroglobulinemia, a progressive syndrome that affects the endothelium (lymphatic system lining). In this disorder, the lymphoma cells produce excessive amounts of an antibody known as immunoglobulin M (IgM). As a result, the abnormally high IgM level makes the blood thick and unable to flow properly. The kidneys may be damaged, and the individual may experience symptoms such as poor vision (due to inadequate circulation in the blood vessels of the eyes), fatigue, easy bleeding, headaches, and dizziness. The disease tends to occur in males more often than in females, and the average age of onset is 63 years. The lymph nodes and spleen frequently are involved, and about 75% of patients also have involvement of the bone marrow. This disease typically is slow growing.
Marginal zone B-cell lymphoma is another lymphoma that tends to progress slowly. If this disease directly affects the lymph nodes, it is called monocytoid B-cell lymphoma; however, if it affects lymphatic tissues at other sites (e.g., stomach, thyroid, skin), it is called mucosa-associated lymphatic tissue, or "MALT" lymphoma. Most low-grade gastric (stomach) lymphomas, and nearly half of all other gastric lymphomas, are marginal zone B-cell lymphomas. Extranodal (outside the lymph nodes) marginal zone tumors may arise in the stomach as well as the lungs, eye sockets, intestines, thyroid, salivary gland, bladder, kidney, and even the central nervous system (CNS). This type of lymphoma affects more women than men, and the average age at diagnosis is 65 years. The majority of patients are diagnosed with localized, early-stage (Stage 1 or 2) extranodal disease. Many patients have a history of autoimmune disease (disease caused by the body's immunologic attacks against its own tissues) such as Sjögren's syndrome or Hashimoto's thyroiditis, or bacterial infection of the stomach with Helicobacter pylori. Research findings suggest that antibiotic therapy for Helicobacter pylori infection may prolong remission in early gastric MALT lymphoma.
Splenic marginal zone lymphomais a rare, slow-growing cancer. It also is known as "splenic lymphoma with villous lymphocytes" - an uncommon form of B-cell chronic lymphocytic leukemia (B-CLL). Splenic marginal zone lymphoma typically involves the spleen and is considered the splenic counterpart of MALT lymphoma. This lymphoma occurs in adults and is slightly more frequent in women than in men. Patients usually have splenomegaly (enlarged spleen) without enlargement of the peripheral lymph nodes. Most patients show bone marrow involvement and modest increases in blood lymphocyte counts.
Adult T-cell leukemia/lymphoma (ATL/L) is a unique cancer that originally was identified in the population of southwestern Japan. It is associated with the retrovirus HTLV-1, which infects the T-cells. ATL/L also is common among Caribbean people, and it has been diagnosed in black residents of the southeastern United States. The average age of diagnosis is 45 years. Patients may exhibit leukemia or generalized enlargement of the lymph nodes. The leukemic form of this disease is more common in Japan, whereas the lymphatic form is more common in the Western hemisphere.
Mycosis fungoides is a rare skin-based (cutaneous) lymphoma that accounts for only 2% to 3% of all NHLs. Skin effects may appear as numerous plaques (flat patches) or nodules (knot-like masses) with/without redness. Sézary syndrome is a variety of this disease that causes widespread reddening of the skin. Lymph node enlargement is uncommon but, when present, usually signals a poor prognosis (disease outcome). This lymphoma is most often diagnosed in older adults (age 55 to 60 years), and men are twice as likely to be affected as women. The cause of mycosis fungoides is unknown.
Large granular lymphocyte leukemia (LGL) is a cancer that is related to T-cell chronic lymphocytic leukemia (T-CLL). In general, LGL is not considered with other cases of malignant lymphoma.
Moderately Aggressive (Intermediate Grade) Lymphomas
Moderately aggressive lymphomas are subdivided into B-cell and T-cell subtypes.
| B-cell |
Diffuse mixed (small and large) cell lymphoma Mantle cell lymphoma Follicle lymphoma (large cell)
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| T-cell |
T-cell chronic lymphocytic leukemia/prolymphocytic leukemia (T-CLL/PLL) Adult T-cell leukemia/ lymphoma (ATL/L) [chronic] Angiocentric lymphoma Angioimmunoblastic lymphoma |
Diffuse large cell lymphoma is the most common type of lymphoma, accounting for approximately one-third of all NHLs, regardless of grade. Patients typically report a history of a rapidly enlarging, symptomatic mass. Approximately two-thirds of all patients have widespread disease at diagnosis, and about 30% of patients also suffer from B symptoms. Prognosis tends to be strongly associated with the International Prognostic Index (IPI) score.
Mantle cell lymphoma is a tumor of the mantle cells that surround the germinal (reproducing) centers of the lymph nodes. Mantle cell lymphoma occurs Primarily in men (approximately 75%), and with an average patient age of 63, it rarely is seen in young patients. At the time of diagnosis, most patients show generalized lymph node disease, with or without enlargement of the spleen and liver. In addition, approximately two-thirds of all newly diagnosed patients have bone marrow involvement. Only about 20% of patients have Stage 1 or 2 disease at diagnosis. The IPI score accurately predicts how most patients will do over time. Although this tumor is a small, B-cell lymphoma, it does not act like one - it is not slow growing and low-grade in character. Instead, the prognosis (predicted outcome) of mantle cell lymphoma is considerably poorer than what was initially expected. The reasons for this inconsistency are unclear, and so there is ongoing investigation into the biology of this disease. The clinical course of mantle cell lymphoma is intermediate between low-grade lymphomas and poorly responsive intermediate-grade lymphomas.
Adult T-cell leukemia/lymphoma (ATL/L) is seen less frequently in its chronic form. In chronic cases of ATL/L, the most noteworthy symptom is skin rash.
Angiocentric lymphoma is a type of T-cell lymphoma that is associated with Epstein Barr virus (EBV) infection. It includes cancers that previously were labeled "lethal midline granulomas," "nasal T-cell lymphoma," and "lymphatoid granulomatosis." Angiocentric lymphoma typically is characterized by a destructive nasal or midline facial tumor that is accompanied by invasive infiltrate, a substance that penetrates the spaces between tissues. The patient's palate (roof of mouth) may be destroyed by the tumor, and he or she may have prominent swelling of the eyes and face. Other sites of involvement outside the lymph nodes (extranodal) are the skin, lungs, and central nervous system. This tumor often is very resistant to therapy and has a poor prognosis. It is much more common in Asians than in people of European descent.
Angioimmunoblastic T-cell lymphoma (AILD) initially was thought to represent an abnormal reaction of the immune system. Now it is considered a variety of T-cell lymphoma. This malignancy usually occurs in adults. Many patients have generalized lymphatic disease (lymphadenopathy), hypergammaglobinemia (increased antibody proteins in the blood), and marked symptoms, including fever, weight loss, and skin rash. Although steroid therapy initially is beneficial in many patients, the disease usually progresses (e.g., to high-grade T-cell immunoblastic lymphoma).
Aggressive and Very Aggressive (High Grade) Lymphomas
Small noncleaved cell lymphoma (SNCL) is a classification that, in general, has been replaced by the terms Burkitt's lymphoma and Burkitt-like (or non-Burkitt's) lymphoma. In children, most SNCLs are Burkitt's lymphomas, whereas most adult SNCLs are Burkitt-like lymphomas.
Burkitt's lymphoma tends to occur most often in children and in adults with compromised immune systems. There are three distinct forms of this disease: endemic, sporadic, and immunodeficient. Endemic Burkitt's lymphoma is seen among the young of equatorial Africa. It frequently affects the jaws of children with developing molar teeth; experts suggest that growth factors may be responsible for the site-specific nature of this tumor. Sporadic Burkitt's lymphoma often involves the abdomen, and it spreads to the bone marrow in about 20% of patients. Immunodeficient Burkitt's lymphoma affects patients with abnormal immune systems (e.g., patients with HIV).
When Burkitt's lymphoma involves the abdomen, it can cause symptoms such as abdominal pain, swelling, nausea, vomiting, and changes in bowel habits. It is a highly malignant lymphoma, but it is potentially curable with very aggressive therapy.
Patients with Burkitt-like lymphoma usually are treated as if they had classic Burkitt's lymphoma.
Large cell immunoblastic lymphoma is considered to be a more aggressive form of diffuse large cell lymphoma, which is categorized as "intermediate" in nature. At diagnosis, this lymphoma is usually a systemic (whole-body) disease. Immunoblastic lymphoma is rapidly fatal if untreated, but frequently curable with intensive chemotherapy.
Lymphoblastic lymphoma often arises in young men, showing high rates around the 16-year and 40-year age groups. In women, there is a more even distribution of disease across all ages. Lymphoblastic lymphoma has several clinical hallmarks, including the production of a mediastinal (middle chest) mass, involvement of the bone marrow and central nervous system (CNS), and "leukemia-like" characteristics similar to adult T-cell leukemia/ lymphoma (T-ALL). But lymph node disease (lymphadenopathy) prevails in lymphomblastic lymphoma, whereas peripheral blood changes prevail in T-ALL. Lymphatic involvement usually occurs above the diaphragm, affecting the nodes of the neck, collarbone, or underarms. In the past, lymphoblastic lymphoma was associated with a poor prognosis; however, complete remission and long-term survival now can be accomplished with the use of intensive chemotherapy programs.
Non-Hodgkin's Lymphoma (continued...)
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