The B-cell lymphomas are types of

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enl ...

affecting

B cell B cells, also known as B lymphocytes, are a type of white blood cell of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or ...

s. Lymphomas are "blood cancers" in the

lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inc ...

s. They develop more frequently in older adults and in immunocompromised individuals. B-cell lymphomas include both Hodgkin's lymphomas and most non-Hodgkin lymphomas. They are typically divided into low and high grade, typically corresponding to indolent (slow-growing) lymphomas and aggressive lymphomas, respectively. As a generalisation, indolent lymphomas respond 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, with some having a good prospect for a permanent

cure A cure is a substance or procedure that ends a medical condition, such as a medication, a surgical operation, a change in lifestyle or even a philosophical mindset that helps end a person's sufferings; or the state of being healed, or cured. The m ...

.Merck Manual home edition
Non-Hodgkin Lymphomas 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-recurrence. 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

CT of primary B-cell lymphoma left ilium

There are numerous kinds of lymphomas involving B cells. The most commonly used classification system is the WHO classification, a convergence of more than one, older classification systems.

Common

Five account for nearly three out of four patients with non-Hodgkin lymphoma: * Diffuse large B-cell lymphoma (DLBCL) * Follicular lymphoma * Marginal zone B-cell lymphoma (MZL) or mucosa-associated lymphatic tissue lymphoma (MALT) *

Small lymphocytic lymphoma Chronic lymphocytic leukemia (CLL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). Early on, there are typically no symptoms. Later, non-painful lymph node swelling, feeling tired, fever, nig ...

(SLL, also known as chronic lymphocytic leukemia, CLL) * Mantle cell lymphoma (MCL)

Rare

The remaining forms are much less common: * DLBCL variants or sub-types of ** Primary mediastinal (thymic) large B cell lymphoma ** T cell/histiocyte-rich large B-cell lymphoma ** Primary cutaneous diffuse large B-cell lymphoma, leg type (Primary cutaneous DLBCL, leg type) ** EBV positive diffuse large B-cell lymphoma of the elderly ** Diffuse large B-cell lymphoma associated with chronic inflammation ** Fibrin-associated diffuse large B-cell lymphoma ** Primary testicular diffuse large B-cell lymphoma *

Burkitt's lymphoma Burkitt lymphoma is a cancer of the lymphatic system, particularly B lymphocytes found in the germinal center. It is named after Denis Parsons Burkitt, the Irish surgeon who first described the disease in 1958 while working in equatorial Afr ...

* Lymphoplasmacytic lymphoma, which may manifest as Waldenström's macroglobulinemia * Nodal marginal zone B cell lymphoma (NMZL) * Splenic marginal zone lymphoma (SMZL) * Intravascular lymphomas variants ** Intravascular large B-cell lymphoma ** Intravascular NK-cell lymphoma ** Intravascular T-cell lymphoma *

Primary effusion lymphoma Primary effusion lymphoma (PEL) is classified as a diffuse large B cell lymphoma. It is a rare malignancy of plasmablastic cells that occurs in individuals that are infected with the Kaposi's sarcoma-associated herpesvirus (i.e. KSHV/HHV8). Plasm ...

Lymphomatoid granulomatosis Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972. Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymor ...

* Primary central nervous system lymphoma * ALK+ large B-cell lymphoma *

Plasmablastic lymphoma Plasmablastic lymphoma (PBL) is a type of large B-cell lymphoma recognized by the World Health Organization (WHO) in 2017 as belonging to a subgroup of lymphomas termed lymphoid neoplasms with plasmablastic differentiation. The other lymphoid neop ...

Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease is a type of large B-cell lymphoma, recognized in the WHO 2008 classification. It is sometimes called the plasmablastic form of multicentric Castleman disease. It has ...

* B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma * B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma

Other

Additionally, some researchers separate out lymphomas that appear to result from other immune system disorders, such as AIDS-related lymphoma. Classic

Hodgkin's lymphoma Hodgkin lymphoma (HL) is a type of lymphoma, in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the patient's lymph nodes. The condition w ...

and nodular lymphocyte predominant Hodgkin's lymphoma are now considered forms of B-cell lymphoma.

Diagnosis

When a person appears to have a B-cell lymphoma, the main components of a workup (for determining the appropriate therapy and the person's prognosis) are: * Establishing the precise subtype: Initially, an incisional or excisional biopsy is preferred. A core needle biopsy is discouraged except in case a lymph node is not easily accessible. Fine-needle aspiration is only acceptable in selected circumstances, in combination with

immunohistochemistry Immunohistochemistry (IHC) is the most common application of immunostaining. It involves the process of selectively identifying antigens (proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to a ...

and

flow cytometry Flow cytometry (FC) is a technique used to detect and measure physical and chemical characteristics of a population of cells or particles. In this process, a sample containing cells or particles is suspended in a fluid and injected into the fl ...

. * Determining the extent of the disease (localized or advanced; nodal or extranodal) * The person's general health status.

Associated chromosomal translocations

Chromosomal translocation In genetics, chromosome translocation is a phenomenon that results in unusual rearrangement of chromosomes. This includes balanced and unbalanced translocation, with two main types: reciprocal-, and Robertsonian translocation. Reciprocal translo ...

s involving the

immunoglobulin heavy locus Immunoglobulin heavy locus, also known as IGH, is a region on human chromosome 14 that contains a gene for the heavy chains of human antibodies (or immunoglobulins). Immunoglobulins recognize foreign antigens and initiate immune responses such ...

is a classic cytogenetic abnormality for many B-cell lymphomas, including follicular lymphoma, mantle cell lymphoma and

. In these cases, the immunoglobulin heavy locus forms a

fusion protein Fusion proteins or chimeric (kī-ˈmir-ik) proteins (literally, made of parts from different sources) are proteins created through the joining of two or more genes that originally coded for separate proteins. Translation of this ''fusion gene'' r ...

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

and mantle cell lymphoma, the other protein in the fusion is

c-myc ''Myc'' is a family of regulator genes and proto-oncogenes that code for transcription factors. The ''Myc'' family consists of three related human genes: ''c-myc'' ( MYC), ''l-myc'' ( MYCL), and ''n-myc'' ( MYCN). ''c-myc'' (also sometimes re ...

(on chromosome 8) and cyclin D1 (on chromosome 11), respectively, which gives the

pro-proliferative ability. In follicular lymphoma, the fused protein is

Bcl-2 Bcl-2 (B-cell lymphoma 2), encoded in humans by the ''BCL2'' gene, is the founding member of the Bcl-2 family of regulator proteins that regulate cell death ( apoptosis), by either inhibiting (anti-apoptotic) or inducing (pro-apoptotic) apoptosi ...

(on chromosome 18), which gives the fusion protein anti-apoptotic abilities.

References

External links

{{Hematological malignancy histology Lymphoma