Non-Hodgkin's lymphoma

Non-Hodgkin's 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 histological subtype, location of lymphoma, and disease stage; symptom severity informs decisions about when to start therapy.

CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) 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 biological 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 Aetiology (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 leukaemia/small lymphocytic lymphoma (see Chronic lymphocytic leukaemia)

• Hairy cell leukaemia (see Hairy cell leukaemia)

• Waldenström's macroglobulinaemia (see Waldenström's macroglobulinaemia)

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