Table 1.
Characteristics of the main B cell malignancies.
| B Cell Malignancy | Pathophysiology | Expression of BCL2 Prosurvival Proteins | Active (Pre-)BCR Signaling | Surface Markers That Are Strongly Expressed | References |
|---|---|---|---|---|---|
| B-ALL | Transformation and expansion of lymphatic B progenitor cells | Bcl-2, Mcl-1, Bcl-xL | pre + | CD19 CD20 CD22 CXCR4 |
[7,35,36,37,38,39,40,41] |
| HCL | Accumulation of CD5+ B cells most in the blood, bone marrow and spleen | Bcl-2 > Mcl-1 | + | CD19 CD20 CD22 CD38 CXCR4 |
[2,11,40,42,43] |
| CLL | Accumulation of CD5+ B cells primarily in the blood and bone marrow | High Bcl-2 > Mcl-1 >> Bcl-xL | + | CD19 CD20 CD38 (poor prognosis) BAFF-R ROR1 CXCR4 |
[3,7,36,38,40,41,44,45] |
| SLL (B-NHL) |
Accumulation of CD5+ B cells most in the lymph nodes | Bcl-2, Mcl-1, Bcl-xL |
+ | CD19 CD20 CD38 (poor prognosis) ROR1 |
[40,44,45,46,47] |
| MM | Accumulation of clonal, Ig secreting plasma cells in the bone marrow | High Bcl-2, Mcl-1, Bcl-xL, Bcl-w | + | CD19 CD22 CD38 BCMA CD13 ADAM17 ROR1 CXCR4 |
[3,7,40,41,48,49,50,51] |
| FL (B-NHL) |
Extensive proliferation and accumulation of abnormal B cells in lymph nodes | High Bcl-2, Bcl-w and Bcl-xL > Mcl-1 | −/+ | CD19 CD20 CD22 CD38 CXCR4 |
[3,7,40,41,45,48,52] |
| MCL (B-NHL) |
Development of abnormal B cells in the mantle zone of lymph nodes, spleen, bone marrow, blood, and gastrointestinal tract | High Bcl-w and Bcl-2 > Mcl-1 | + | CD19 CD20 CD22 CD38 ROR1 CXCR4 |
[3,7,40,41,47,52,53] |
| MZL (B-NHL) |
Development of abnormal B cells in the marginal zones of lymph tissue | High Bcl-2 and Bcl-w > Bcl-xL | + | CD19 CD20 CD22 CXCR4 |
[3,10,40,41,52,54,55] |
| DLBCL (B-NHL) |
Development of abnormal B cells in germinal centers of secondary lymphoid organs | Bcl-2 and Bcl-w > Mcl-1, Bcl-xL | + | CD19 CD20 CD22 CD38 CXCR4 |
[3,7,36,40,41,45,52,56,57] |
| WM (B-NHL) |
Proliferation of clonal, IgM-secreting plasma cells in the lymph nodes and bone marrow | High Bcl-2 > Bcl-xL and Mcl-1 | + | CD19 CD20 CD38 CXCR4 (mutation) |
[8,40,46,58,59] |
ADAM17, a disintegrin and metalloprotease 17; B-ALL, B-acute lymphoblastic leukemia; BAFF-R, B-cell activating factor receptor; BCMA, B cell maturation antigen; BCR, B-cell receptor; B-NHL, B cell non-Hodgkin lymphoma; CLL, chronic lymphocytic leukemia; CXCR4, CXC chemokine receptor 4 specific for stromal cell-derived factor-1. DLBCL, diffuse large B cell lymphoma; FL, follicular lymphoma; HCL, hairy cell leukemia; MCL, mantle cell lymphoma; MM, multiple myeloma; MZL, marginal zone lymphoma; ROR1, receptor tyrosine kinase-like orphan receptor; SLL, small lymphocytic lymphoma; WM, Waldenström’s macroglobulinemia. Present on precursor B lymphocytes, pre-BCR differs from conventional BCRs in that it possesses a germ line-encoded surrogate light chain which is associated with the signal transduction machinery via heavy chain proteins that have been generated by productive rearrangement of the immunoglobulin HC genes.