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B-cell lymphoma

Abstract

The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. 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.

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

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.

Types | 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 (also known as chronic lymphocytic leukemia, CLL)

- Mantle cell lymphoma (MCL)

Types | 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 inflammation

- Burkitt's lymphoma

- Lymphoplasmacytic lymphoma, which may manifest as Waldenström's macroglobulinemia

- Nodal marginal zone B cell lymphoma (NMZL)

- Splenic marginal zone lymphoma (SMZL)

- Intravascular large B-cell lymphoma

- Primary effusion lymphoma

- Lymphomatoid granulomatosis

- Primary central nervous system lymphoma

- ALK-positive large B-cell lymphoma

- Plasmablastic lymphoma

- Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease

- 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

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

Associated chromosomal translocations

Chromosomal translocations 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.