Non-Hodgkin lymphoma

Non-Hodgkin lymphomas (NHLs) are a heterogeneous group of lymphoid malignancies.

Clinical history and presenting signs/symptoms depend on the type and location of lymphoma, and disease stage at presentation.

Diagnosis is confirmed by histopathology (e.g., biopsy of lymph node or extranodal sites) and immunophenotyping.

Diffuse large B-cell lymphoma is the most common type of NHL.

Treatment varies depending on the histologic subtype, location of lymphoma, and disease stage; symptom severity informs decisions about when to start therapy.

CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) is the most commonly used chemotherapy regimen.

NHLs are a heterogeneous group of lymphoid malignancies that originate from B cells, T cells, or natural killer (NK) cells.[1]Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022 Jul;36(7):1720-48. https://www.nature.com/articles/s41375-022-01620-2 http://www.ncbi.nlm.nih.gov/pubmed/35732829?tool=bestpractice.com [2]Campo E, Jaffe ES, Cook JR, et al. The International Consensus Classification of mature lymphoid neoplasms: a report from the Clinical Advisory Committee. Blood. 2022 Sep 15;140(11):1229-53. https://pmc.ncbi.nlm.nih.gov/articles/PMC9479027 http://www.ncbi.nlm.nih.gov/pubmed/35653592?tool=bestpractice.com ​ These malignancies develop in lymphoid tissue (e.g., lymph nodes, spleen) or other parts of the body (e.g., central nervous system, gastrointestinal tract, liver, skin), depending on the subtype.

There are over 60 subtypes of NHL with distinct biologic and clinical features.[1]Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022 Jul;36(7):1720-48. https://www.nature.com/articles/s41375-022-01620-2 http://www.ncbi.nlm.nih.gov/pubmed/35732829?tool=bestpractice.com [2]Campo E, Jaffe ES, Cook JR, et al. The International Consensus Classification of mature lymphoid neoplasms: a report from the Clinical Advisory Committee. Blood. 2022 Sep 15;140(11):1229-53. https://pmc.ncbi.nlm.nih.gov/articles/PMC9479027 http://www.ncbi.nlm.nih.gov/pubmed/35653592?tool=bestpractice.com ​ See Etiology (Classification).

This topic covers management of the main subtypes of B-cell lymphoma (i.e., diffuse large B-cell lymphoma, mantle cell lymphoma, follicular lymphoma, marginal zone lymphoma [nodal and splenic], primary central nervous system lymphoma) and NK/T-cell lymphoma (e.g., peripheral T-cell lymphomas).

This topic does not cover the following NHLs:

• Mycosis fungoides/Sézary syndrome (see  Cutaneous T-cell lymphoma)

• Chronic lymphocytic leukemia/small lymphocytic lymphoma (see  Chronic lymphocytic leukemia)

• Hairy cell leukemia (see  Hairy cell leukemia)

• Waldenström macroglobulinemia (see Waldenström macroglobulinemia)

• Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (see MALT lymphoma)