What is the WHO classification of mature B-cell neoplasms in B-cell lymphoma?

Updated: Feb 23, 2021
  • Author: Mohammad Muhsin Chisti, MD, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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The following classification deals with only the mature B-cell neoplasms. Both lymphomas and lymphoid leukemias are included in this classification because both solid and circulating phases are present in many lymphoid neoplasms and distinction between them is artificial. For instance, B-cell chronic lymphocytic leukemia (CLL) and B-cell small lymphocytic lymphoma (SLL) are different manifestations of the same neoplasm, as are lymphoblastic lymphomas and T-cell acute lymphocytic leukemias.

The WHO classification of mature B-cell neoplasms is as follows [3] :

  • Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)
  • Monoclonal B-cell lymphocytosis
  • B-cell prolymphocytic leukemia
  • Splenic marginal zone lymphoma
  • Hairy cell leukemia
  • Splenic B-cell lymphoma/leukemia, unclassifiable
  • Splenic diffuse red pulp small B-cell lymphoma
  • Hairy cell leukemia–variant
  • Lymphoplasmacytic lymphoma
  • Waldenström macroglobulinemia
  • Monoclonal gammopathy of undetermined significance (MGUS), IgM
  • Heavy-chain diseases (μ, γ, α)
  • MGUS, IgG/A
  • Plasma cell myeloma
  • Solitary plasmacytoma of bone
  • Extraosseous plasmacytoma
  • Monoclonal immunoglobulin deposition diseases
  • Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
  • Nodal marginal zone lymphoma
  • Pediatric nodal marginal zone lymphoma
  • Follicular lymphoma
  • In situ follicular neoplasia
  • Duodenal-type follicular lymphoma
  • Pediatric-type follicular lymphoma
  • Large B-cell lymphoma with  IRF4 rearrangement
  • Primary cutaneous follicle center cell lymphoma
  • Mantle cell lymphoma
  • In situ mantle cell neoplasia
  • Diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS)
  • Germinal center B-cell type
  • Activated B-cell type
  • T-cell/histiocyte-rich large B-cell lymphoma
  • Primary DLBCL of the central nervous system (CNS)
  • Primary cutaneous DLBCL, leg type
  • Epstein-Barr virus (EBV)–positive DLBCL, NOS
  • EBV-positive mucocutaneous ulcer
  • DLBCL associated with chronic inflammation
  • Lymphomatoid granulomatosis
  • Primary mediastinal (thymic) large B-cell lymphoma
  • Intravascular large B-cell lymphoma
  • Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma
  • Plasmablastic lymphoma
  • Primary effusion lymphoma
  • Human herpesvirus 8 (HHV-8)–associated DLBCL, NOS
  • Burkitt lymphoma
  • Burkitt-like lymphoma with 11q aberration
  • High-grade B-cell lymphoma with  MYC and  BLC2 and/or  BCL6 rearrangements
  • High-grade B-cell lymphoma, NOS
  • B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma

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