Mature B-Cell Neoplasms (general)

Definition

B-cell lymphoproliferative disorders are a clonal expansion of the various stages of  B lymphocytes in bone marrow, blood or other tissues.

Epidemiology

Mature B cell neoplasms comprise over 90% of lymphoid neoplasms worldwide and there are 4% of nw cancers each year. They are more common in developing countries. The most common types are follicular lymphoma (FL) and diffuse large B-cell lymphoma which make up 50% of the non-Hodgkin's lymphomas. The specific B-cell neoplasms frequency vary in different parts of the world. FL is more common in the US and Western Europe. Burkitt's lymphoma in endemic in Africa.

Causes

Infectious agents have been shown to contribute to the development of several types of B-cell neoplasms. EBV virus is present in almost 100% of BL. HHV8/karposi Sarcoma herpesvirus (KSHV)  is associated with primary effusion lymphoma (PEL). Some bacteria/immune suppression due to infection has been implicated in MALT lymphoma.  H. Pylori has been seen in progression to lymphoma.

Classification

The following is the WHO classification of Mature B-lymphoid neoplasms; Case types in bold are more commonly found.

• B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma
• B-cell prolymphocytic leukemia
• Lymphoplasmacytic lymphoma
• Splenic marginal zone B-cell lymphoma 
• Hairy cell leukemia 
• Plasma cell myeloma/plasmacytoma
• Extranodal marginal zone B-cell lymphoma of MALT type
• Nodal marginal zone B-cell lymphoma
• Follicular lymphoma
• Mantle-cell lymphoma
• Diffuse large B-cell lymphoma
• Burkitt’s lymphoma

Immunophenotyping

Below is a table of the immunophenotypic profiles of the major B-cell lymphoproliferations:

 * = lymphoplasmacytic lymphoma

**  = plasma cell myeloma             

Other relevant tests

Flow Diagnosis: To diagnose  see individual case type

References