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B-cell lymphoma

Blood cancer that affects B-type white blood cells

Field	Value
name	B-cell lymphoma
image	Large b cell lymphoma - cytology small.jpg
caption	Micrograph showing a large B cell lymphoma. Field stain.
field	Hematology, oncology

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.

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 (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 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
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
Lymphomatoid granulomatosis
Primary central nervous system lymphoma
ALK+ 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

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.

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

Follicular lymphoma	Marginal zone B-cell lymphoma (MZL) or mucosa-associated lymphatic tissue (MALT) lymphoma	Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL)	Mantle cell lymphoma (MCL)
CD5	-	-	+
CD10	+	-	-
CD23	-	-	+
Cyclin D1	-	-	-

Associated chromosomal translocations

Chromosomal translocations involving the immunoglobulin heavy locus 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.

References

[http://www.merck.com/mmhe/sec14/ch177/ch177c.html Merck Manual home edition], Non-Hodgkin Lymphomas
(2017). "Primary B-cell lymphoma of the pelvic bone in a young patient: Imaging features of a rare case". Cancer Research Frontiers.
(May 2006). "The Lymphomas". The Leukemia & Lymphoma Society.
Mazen Sanoufa. (2010). "B-Cell Lymphoma of the Thoracic Spine Presenting with Spinal Cord Pressure Syndrome". Journal of Clinical Medicine Research.
"HMDS: Hodgkin's Lymphoma".
Mohammad Muhsin Chisti, Haresh Kumar, Sumeet K Yadav. "B-Cell Lymphoma Workup".
(2018). "Practical Pathology of Serous Membranes".
(10 September 2001). "Mechanisms of chromosomal translocations in B cell lymphomas.". Oncogene.
(May 1999). "Detection of translocation t(11;14)(q13;q32) in mantle cell lymphoma by fluorescence in situ hybridization". Am. J. Pathol..

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