Glossary

Reed-Sternberg cells ^

Also known as lacunar histiocytes for certain types, are different giant cells found with light microscopy in biopsies from individuals with Hodgkin’s lymphoma (aka Hodgkin’s disease; a type of lymphoma).

They are usually derived from B-lymphocytes classically considered crippled germinal center B cells, meaning they have not undergone hypermutation to express their antibody. Seen against a sea of B cells, they give the tissue a moth-eaten appearance.

They are named after Dorothy Reed Mendenhall and Carl Sternberg, who provided the first definitive microscopic descriptions of Hodgkin’s disease.

Classification of Hodgkin’s is based on the reactive cell mixture. Immunomarkers are used e.g. CD15 and 30.

Reed-Sternberg cells are large and are either multinucleated or have a bilobed nucleus with prominent eosinophilic inclusion-like nucleoli (thus resembling an “owl’s eye” appearance).

A special type of Reed-Sternberg cells (RSCs) is the lacunar histiocyte, whose cytoplasm retracts when fixed in formalin, so the nuclei give the appearance of cells that lie with empty spaces (called lacunae) between them. These are characteristic of the nodular sclerosis subtype of Hodgkin’s lymphoma.

AIDS-Related Lymphomas ^

Lymphomas occurring in HIV-positive patients are usually aggressive. It is estimated that as many as ten percent of people who are HIV-positive will ultimately develop lymphoma. Although both Hodgkin and non-Hodgkin lymphomas may occur in AIDS patients, non-Hodgkin lymphomas are more common and include diffuse large B-cell, Burkitt’s/Burkitt-like and primary central nervous system lymphoma.

Anaplastic Large-Cell Lymphoma ^

Anaplastic large-cell lymphoma (ALCL) is a rare type of aggressive T-cell lymphoma comprising about ? percent of all lymphomas in adults and between ? percent and ? percent of all lymphomas in children.

Angioimmunoblastic Lymphoma ^

Angioimmunoblastic lymphoma (AILD) is a fast-growing T-cell lymphoma that accounts for between ? percent and ? percent of all cases of NHL on the island of Ireland

Blastic NK-Cell Lymphoma ^

Blastic NK-cell lymphoma is a very rare T-cell lymphoma, affecting only a few people (usually adults) each year. This lymphoma is very fast growing, is difficult to treat and can arise anywhere in the body. Since this disease is so rare, patients should consult with their medical team to find promising therapies or clinical trials.

Burkitt’s Lymphoma, Burkitt-like Lymphoma (Small Non-Cleaved Cell Lymphoma) ^

Burkitt’s lymphoma is an aggressive B-cell form of NHL that occurs most often in children and young adults. There are three main types of Burkitt’s lymphoma: sporadic, endemic and immunodeficiency-related disease. While sporadic Burkitt’s lymphoma occurs throughout most of the world, endemic Burkitt’s lymphoma is found mostly in Africa and is often associated with the Epstein-Barr virus (EBV). Immunodeficiency-related Burkitt’s lymphoma is diagnosed most often in people infected with HIV/AIDS. The disease may affect the jaw, central nervous system, bone marrow, bowel, kidneys, ovaries or other organs. Burkitt’s lymphoma is potentially curable.

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma ^

According to the Irish Cancer Society, approximately ?? new cases of CLL and ?? new cases of SLL are diagnosed annually.

Cutaneous T-Cell Lymphoma ^

Cutaneous T-cell lymphomas (CTCL) arise in the skin and account for approximately ?? percent of all NHL cases..

Diffuse Large B-Cell Lymphoma ^

Diffuse large B-cell lymphoma (DLBCL) is the most common form of NHL, accounting for up to ??? of newly diagnosed cases.

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. As with other rare cancers, patients should discuss treatment options with their medical team.

Follicular Lymphoma ^

Follicular lymphoma is a relatively common lymphoma, making up between 20 percent and 30 percent of all NHLs, and typically occurs in middle-aged and older adults, but it can affect younger people in their 30s and 40s.

Hepatosplenic Gamma-Delta 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.

Lymphoblastic Lymphoma ^

Lymphoblastic lymphoma can appear in both B-cells and T-cells, but is much more common in T-cells, comprising ?? percent of all lymphoblastic lymphomas. This lymphoma is most often diagnosed in children. With intensive chemotherapy, the complete remission rate can be very high.

Mantle Cell Lymphoma ^

Mantle cell lymphoma is a B-cell lymphoma that affects approximately ?? percent of all NHL patients.

Marginal Zone Lymphoma ^

Marginal zone B-cell lymphomas, a group of indolent lymphomas whose cells come from B-lymphocytes normally found in the marginal zone of the secondary lymphoid follicles in the spleen and lymph nodes, accounts for approximately ?? percent of all NHLs.

The median age for diagnosis of this type of lymphoma is 65.

Marginal zone lymphomas encompass three basic types:

(1) Extranodal or mucosa-associated lymphoid tissue (MALT), occurring outside the lymph nodes

(2) Nodal, occurring within the lymph nodes

(3) splenic, occurring mostly in the spleen and blood.

Skin-associated lymphoid-tissue-related B-cell lymphoma (SALT) is also considered a form of MALT lymphoma.

Marginal zone and MALT lymphomas vary from other types of B-cell NHLs in a number of ways:

(1) Their natural history is different

(2) Many people who develop MALT lymphoma have a history of inflammation or autoimmune disorders

(3) Chronic inflammation is associated with Helicobacter pylori (H. pylori), a microbial pathogen linked to chronic gastritis

(4) Sometimes, MALT lymphomas can be treated with antibiotics.

Nasal T-Cell Lymphoma ^

Although this fast-growing lymphoma is very rare in Ireland, it is relatively common in Asia and parts of Latin America, leading researchers to suspect that some ethnic groups may be more prone to this cancer, which affects both children and adults. This type of lymphoma is associated with the Epstein-Barr virus. As with other rare cancers, patients should consult with their medical team for treatment options and the availability of clinical trials.

Pediatric Lymphoma ^

Childhood NHL comprises about ?? percent of all NHL cases diagnosed in Ireland. The most common types are lymphoblastic lymphoma, Burkitt’s lymphoma, diffuse large B-cell lymphoma and anaplastic large-cell lymphoma. Lymphoblastic lymphoma is closely related to childhood acute lymphoblastic leukemia. The number of children with NHL continues to increase.

Peripheral T-Cell Lymphomas ^

Peripheral T-cell lymphomas (PTCL) refer to a large number of different T-cell lymphomas that together comprise between 10 percent and 15 percent of all NHL cases and can occur anytime during adulthood.

Primary Central Nervous System Lymphoma ^

Primary central nervous system lymphoma (PCNSL) is a type of cancer that is limited to the brain or spinal cord but may also be found in tissues around the eye. An increasing occurrence of this disease has been seen in patients with AIDS and others whose immune system has been compromised. Median age of diagnosis for patients with PCNSL is 55 years for patients with a normal immune system and 31 years for AIDS patients. Although in the past the outlook for patients with this cancer has been poor, today, the survival rate has greatly improved.

T-Cell Leukemias ^

T-cell leukemias are also derived from T-cells and can act like T-cell lymphoma. These cancers include T-cell promyelocytic leukemia, T-cell granular lymphocytic leukemia, aggressive NK-cell leukemia and adult T-cell lymphoma/leukemia.

Transformed Lymphomas ^

Although indolent B-cell lymphomas, such as follicular lymphomas, are most commonly associated with transforming to aggressive disease, slow-growing T-cell lymphomas can also progress to aggressive disease.

Treatment-Related T-Cell Lymphomas ^

Treatment-related T-cell lymphomas may appear after solid organ or bone marrow transplantation. The immune system suppression that is required for transplant patients can put them at risk for developing post-transplant lymphoproliferative disorders, certain unusual forms of peripheral T-cell lymphoma and other types of NHL. Treatment-related T-cell lymphomas may require therapy that differs from the standard treatments normally used to treat these conditions.

Waldenström Macroglobulinemia ^

Waldenström Macroglobulinemia (also known as lymphoplasmacytic lymphoma or immunocytoma) is a rare B-cell lymphoma that occurs in less than two percent of people with NHL.