B-cell lymphoma
Encyclopedia
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 (such as those with AIDS
).
B-cell lymphomas include both Hodgkin's lymphoma
s and most non-Hodgkins lymphomas. They are often divided into indolent (slow-growing) lymphomas and aggressive lymphomas. Indolent lymphomas respond rapidly to treatment and are kept under control (in remission) with long-term survival of many years, but are not cured. Aggressive lymphomas usually require intensive treatments, but have good prospects for a permanent cure
.
Prognosis and treatment depends on the specific type of lymphoma as well as the stage and grade. Treatment includes radiation and chemotherapy. Early-stage indolent B-cell lymphomas can often be treated with radiation alone, with long-term non-reoccurrence. Early-stage aggressive disease is treated with chemotherapy and often radiation, with a 70-90% cure rate. Late-stage indolent lymphomas are sometimes left untreated and monitored until they progress. Late-stage aggressive disease is treated with chemotherapy, with cure rates of over 70%.
.
Classic Hodgkin's lymphoma
and nodular lymphocyte predominant Hodgkin's lymphoma
are now considered forms of B-cell lymphoma.
s involving the immunoglobulin heavy locus (IGH@) is a classic cytogenetic abnormality for many B-cell lymphomas, including follicular lymphoma
, mantle cell lymphoma
and Burkitt's lymphoma
. In these cases, The immunoglobulin heavy locus forms a fusion protein
with another protein that has pro-proliferative or anti-apoptotic abilities. The enhancer element of the immunoglobulin heavy locus, which normally functions to make B cells produce massive production of antibodies, now induces massive transcription of the fusion protein, resulting in excessive pro-proliferative or anti-apoptotic effects on the B cells containing the fusion protein. In Burkitt's lymphoma
and mantle cell lymphoma
, the other protein in the fusion is c-myc (on chromosome 8) and cyclin D1
(on chromosome 11), respectively, which gives the fusion protein
pro-proliferative ability. In follicular lymphoma
, the fused protein is
Bcl-2
(on chromosome 18), which gives the fusion protein anti-apoptotic abilities.
Lymphoma
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...
affecting B cells. Lymphomas are "blood cancers"
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...
in the lymph glands. They develop more frequently in older adults and in immunocompromised individuals (such as those with AIDS
AIDS
Acquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
).
B-cell lymphomas include both 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...
s and most non-Hodgkins lymphomas. They are often divided into indolent (slow-growing) lymphomas and aggressive lymphomas. Indolent lymphomas respond rapidly to treatment and are kept under control (in remission) with long-term survival of many years, but are not cured. Aggressive lymphomas usually require intensive treatments, but have good prospects for a permanent cure
Cure
A cure is a completely effective treatment for a disease.The Cure is an English rock band.Cure, or similar, may also refer to:-Film and television:* The Cure , a short film starring Charlie Chaplin...
.
Prognosis and treatment depends on the specific type of lymphoma as well as the stage and grade. Treatment includes radiation and chemotherapy. Early-stage indolent B-cell lymphomas can often be treated with radiation alone, with long-term non-reoccurrence. Early-stage aggressive disease is treated with chemotherapy and often radiation, with a 70-90% cure rate. Late-stage indolent lymphomas are sometimes left untreated and monitored until they progress. Late-stage aggressive disease is treated with chemotherapy, with cure rates of over 70%.
Types
There are fourteen kinds of lymphomas involving B cells.Common
Five account for nearly three out of four patients with non-Hodgkin lymphoma:- 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...
- 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...
- Mucosa-Associated Lymphatic Tissue lymphoma (MALT)
- Small cell lymphocytic lymphoma (overlaps with 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...
) - 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...
(MCL)
Rare
The remaining nine are much less common:- Burkitt lymphoma
- Mediastinal large B cell lymphomaMediastinal large B cell lymphomaMediastinal large B-cell lymphoma is a form of lymphoma involving the mediastinum.Types include:*Primary mediastinal B-cell lymphoma - affects primarily young adults*Diffuse large B cell lymphoma...
- 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,"...
- 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) - 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,...
(SMZL) - Intravascular large B-cell lymphomaIntravascular large B-cell lymphomaIntravascular large B-cell lymphoma, abbreviated ILBCL and also referred to as angiotropic large-cell lymphoma, intralymphatic lymphomatosis, intravascular lymphomatosis,, and, less specifically intravascular lymphoma.-Diagnosis:...
- 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 ....
- Lymphomatoid granulomatosisLymphomatoid granulomatosisLymphomatoid granulomatosis is a neoplastic disease.It is a lymphoproliferative disorder . The word granulomatosis denotes one of its microscopic character, polymorphic lymphoid infiltrates and focal necrosis within it....
Other
Additionally, some researchers separate out lymphomas that appear result from other immune system disorders, such as AIDS-related lymphomaAIDS-related lymphoma
AIDS-related lymphoma describes lymphomas occurring in patients with acquired immunodeficiency syndrome .A lymphoma is a type of cancer arising from lymphoid cells...
.
Classic 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...
and nodular lymphocyte predominant Hodgkin's lymphoma
Nodular lymphocyte predominant Hodgkin's lymphoma
Nodular lymphocyte predominant Hodgkin's lymphoma is a form of lymphoma.It is no longer classified as a form of classic Hodgkin's lymphoma...
are now considered forms of B-cell lymphoma.
Associated chromosomal translocations
Chromosomal translocationChromosomal 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...
s involving the immunoglobulin heavy locus (IGH@) is a classic cytogenetic abnormality for many B-cell lymphomas, including 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...
, 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...
and Burkitt's lymphoma
Burkitt's lymphoma
Burkitt's lymphoma is a cancer of the lymphatic system...
. In these cases, The immunoglobulin heavy locus forms a fusion protein
Fusion protein
Fusion proteins or chimeric proteins are proteins created through the joining of two or more genes which originally coded for separate proteins. Translation of this fusion gene results in a single polypeptide with functional properties derived from each of the original proteins...
with another protein that has pro-proliferative or anti-apoptotic abilities. The enhancer element of the immunoglobulin heavy locus, which normally functions to make B cells produce massive production of antibodies, now induces massive transcription of the fusion protein, resulting in excessive pro-proliferative or anti-apoptotic effects on the B cells containing the fusion protein. In Burkitt's lymphoma
Burkitt's lymphoma
Burkitt's lymphoma is a cancer of the lymphatic system...
and 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...
, the other protein in the fusion is c-myc (on chromosome 8) and 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:...
(on chromosome 11), respectively, which gives the fusion protein
Fusion protein
Fusion proteins or chimeric proteins are proteins created through the joining of two or more genes which originally coded for separate proteins. Translation of this fusion gene results in a single polypeptide with functional properties derived from each of the original proteins...
pro-proliferative ability. In 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...
, the fused protein is
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...
(on chromosome 18), which gives the fusion protein anti-apoptotic abilities.