Chapter 30: Non-Hodgkin Lymphoma

Pathology (WHO 2016)

Mature B-cell neoplasms

• Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

• 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^*

• μ heavy-chain disease

• γ heavy-chain disease

• α heavy-chain disease

• Monoclonal gammopathy of undetermined significance (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 lymphoma

• Mantle cell lymphoma

  • In situ mantle cell neoplasia^*

• Diffuse large B-cell lymphoma (DLBCL), 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

• EBV+ DLBCL, NOS^*

• EBV+ mucocutaneous ulcer^*†

• DLBCL associated with chronic inflammation

• Lymphomatoid granulomatosis

• Primary mediastinal (thymic) large B-cell lymphoma

• Intravascular large B-cell lymphoma

• ALK+ large B-cell lymphoma

• Plasmablastic lymphoma

• Primary effusion lymphoma

• HHV8+ DLBCL, NOS^*†

• Burkitt lymphoma

• Burkitt-like lymphoma with 11q aberration^*†

• High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements^*

• High-grade B-cell lymphoma, NOS^*

• B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classic Hodgkin lymphoma

Mature T And NK Neoplasms

• T-cell prolymphocytic leukemia

• T-cell large granular lymphocytic leukemia

• Chronic lymphoproliferative disorder of NK cells^†

• Aggressive NK-cell leukemia

• Systemic EBV+ T-cell lymphoma of childhood^*

• Hydroa vacciniforme–like lymphoproliferative disorder^*

• Adult T-cell leukemia/lymphoma

• Extranodal NK-/T-cell lymphoma, nasal type

• Enteropathy-associated T-cell lymphoma

• Monomorphic epitheliotropic intestinal T-cell lymphoma^*

• Indolent T-cell lymphoproliferative disorder of the GI tract^*†

• Hepatosplenic T-cell lymphoma

• Subcutaneous panniculitis-like T-cell lymphoma

• Mycosis fungoides

• Sézary syndrome

• Primary cutaneous CD30+ T-cell lymphoproliferative disorders

  • Lymphomatoid papulosis

  • Primary cutaneous anaplastic large cell lymphoma

• Primary cutaneous γδ T-cell lymphoma

• Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma^†

• Primary cutaneous acral CD8+ T-cell lymphoma^*†

• Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder^*†

• Peripheral T-cell lymphoma, NOS

• Angioimmunoblastic T-cell lymphoma

• Follicular T-cell lymphoma^*†

• Nodal peripheral T-cell lymphoma with TFH phenotype^*†

• Anaplastic large-cell lymphoma, ALK+

• Anaplastic large-cell lymphoma, ALK-^*

• Breast implant–associated anaplastic large-cell lymphoma^*†

Posttransplant Lymphoproliferative Disorders (PTLD)

• Plasmacytic hyperplasia PTLD

• Infectious mononucleosis PTLD

• Florid follicular hyperplasia PTLD^*

• Polymorphic PTLD

• Monomorphic PTLD (B- and T-/NK-cell types)

• Classic Hodgkin lymphoma PTLD

Histiocytic and Dendritic Cell Neoplasms

• Histiocytic sarcoma

• Langerhans cell histiocytosis

• Langerhans cell sarcoma

• Indeterminate dendritic cell tumor

• Interdigitating dendritic cell sarcoma

• Follicular dendritic cell sarcoma

• Fibroblastic reticular cell tumor

• Disseminated juvenile xanthogranuloma

• Erdheim-Chester disease^*

^*Changes from the WHO 2008 classification

^†Provisional entity

Swerdlow SH et al, eds. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th Edition. Lyon, France: IARC ...