Non-Hodgkin Lymphoma (NHL)

Background

• Non-Hodgkin lymphoma (NHL) is a heterogeneous family of lymphoid malignancies, which typically develop in lymph nodes but may occur in almost any tissue.
• Most cases (85%-90%) of NHL are of B-cell origin, with 10%-15% derived from T cells or NK cells.
• There are 61 subtypes of NHL recognized in the 2016 World Health Organization classification.
• The global incidence of NHL is 6.7 per 100,000 persons in men and 4.7 per 100,000 persons in women, with incidence increasing with age.
• The disease is varied in its course depending on subtype and ranges from indolent (follicular lymphoma) to aggressive (diffuse large B-cell lymphoma or Burkitt lymphoma).
• Immunosuppression is the most well-established risk factor for NHL; some viruses such as HIV are reported to be associated with particular subtypes of NHL.

Evaluation

• Clinical presentation can be varied depending on the site of involvement, presence of B-symptoms (weight loss > 10%, night sweats, and fever), and subtype of NHL.
• Suspect a diagnosis of NHL in patients who present with painless, persistent generalized lymphadenopathy in a single or in multiple sites with or without B-symptoms.
• Typical testing for the initial diagnosis and staging of NHL includes blood tests, biopsy of lymph nodes and bone marrow, and imaging, as well as immunophenotyping and fluorescence in situ hybridization testing.
• See the Testing Overview of specific lymphoma subtype in the following .topics for details on testing, including
  • Follicular Lymphoma
  • Diffuse Large B-cell Lymphoma
  • Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL)
  • Mantle Cell Lymphoma
  • Mycosis Fungoides/Sezary Syndrome
  • Hairy Cell Leukemia
  • Marginal Zone Lymphoma
  • MALT Lymphoma (extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue)
  • Adult T-cell Leukemia-lymphoma (ATLL)
  • Waldenstrom Macroglobulinemia

Management

• The management and prognosis for patients with NHL varies greatly depending on the subtype of lymphoma, Ann Arbor stage at presentation, International Prognostic Index (IPI) risk score, and presence or absence of B-symptoms.
• The specific management of each subtype of NHL varies greatly; see the management section of relevant topics.
• Consider enrollment in a clinical trial for all patients with NHL; for a list of trials recruiting patients with NHL, see clinicaltrials.gov.
• Patients with NHL may be at risk of developing complications such as tumor lysis syndrome, superior vena cava syndrome, viral reactivation, and thrombotic events.
• Patients with NHL require long-term follow-up for screening and prevention of additional cancers, cardiovascular disease related to treatments, and/or other survivorship issues.