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B-cell lymphoid neoplasm (B-cell lymphoma)

Also see non-Hodgkin's lymphoma. Classification: 1) indolent a) small lymphocytic lymphoma b) follicular lymphoma c) marginal zone lymphoma d) MALT lymphoma e) splenic lymphoma f) nodal lymphoma 3) aggressive a) diffuse large cell lymphoma b) mantle cell lymphoma 3) highly aggressive a) Burkett lymphoma b) lymphoblastic lymphoma c) AIDS-related lymphoma Genetics: 1) the bcl-7a gene is a site of recurrent breakpoint in high grade B cell lymphomas 2) chromosomal translocation t(8;14;12)(q24.1;q32.3;q24.1) involving BCL7A, MYC & immunoglobulin gene region associated with B-cell non-Hodgkin lymphoma 3) chromosomal translocation t(8;14)(p23.1;q21) involving MFHAS1 with a cryptic exon named '14q21 element' may be a cause of B-cell lymphoma, resulting fusion protein named 'chimeric MASL1' is tumorigenic in nude mice 4) chromosomal translocation t(11;14)(q23;q32) involving DDX6 may be a cause of B-cell lymphoma 5) chromosomal translocation t(3;14)(q27;q32) involving BCL6 with immunoglobulin gene regions may be a cause of B-cell non-Hodgkin lymphoma 6) chromosomal translocation t(3;11)(q27;q23) involving BCL6 with POU2AF1/OBF1 may be a cause of a form of B-cell leukemia 7) other implicated genes MIRH1, BMI1 Laboratory: - see ARUP consult [4] Management: - indolent B-cell lyphomas - follicular lymphoma, CLL, hairy cell leukemia are observed without therapy until symptomatic [3] - rituximab + multiagent chemotherapy when symptomayic vs allogeneic hematopoietic stem cell transplantation - gastric MALT lymphomas are treated with eradication of H pylori [3] - aggressive B-cell lymphomas - diffuse large B-cell lymphoma, mantle cell lymphomas, Burkitt lymphoma - combined chemotherapy including rituximab - prophylaxis for tumor lysis syndrome for Burkitt lymphoma [3]

Interactions

disease interactions

Specific

cutaneous B-cell lymphoma germinal center B-cell lymphoma mature B-cell acute lymphocytic leukemia (ALL) peripheral B-cell lymphoid neoplasm precursor B-cell lymphoid neoplasm small B-cell lymphoma

General

non-Hodgkin's lymphoma

References

  1. Entrez Gene :accession 605
  2. UniProt :accession Q4VC05
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 18. American College of Physicians, Philadelphia 2009, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. ARUP Consult: B-Cell Lymphomas The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/lymphomas-b-cell
  5. NEJM Knowledge+