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NHL Subtypes

More than 60 specific NHL subtypes have been identified and assigned names by the World Health Organization (WHO).  NHL subtypes are categorized by the characteristics of the lymphoma cells, including their appearance, the presence of proteins on the surface of the cells and their genetic features. It's important to know your subtype since it plays a large part in determining the type of treatment you'll receive. A hematopathologist, a doctor who specializes in the diagnosis of blood disorders and blood cancers, should review your biopsy specimens.

Specialists further characterize the NHL subtypes according to how the disease progresses:

  • Aggressive lymphomas are fast-moving and account for about 60 percent of all NHL cases. Diffuse large B-cell lymphoma (DLBCL) is the most common aggressive NHL subtype.
  • Indolent lymphomas are slow-moving and tend to grow more slowly and have fewer signs and symptoms when first diagnosed. Slow-growing or indolent subtypes represent about 40 percent of all NHL cases. Follicular lymphoma (FL) is the most common subtype of indolent NHL. 

The treatments for aggressive and indolent lymphomas are different.  When a patient’s rate of disease progression is between indolent and aggressive, he or she is considered to have “intermediate grade” disease. Some cases of indolent NHL can transform into aggressive NHL.


 

Diagnostic Designations for Non-Hodgkin Lymphoma (NHL): Subtypes

This list provides some of the diagnostic designations for NHL subtypes based on the WHO classification, categorized by cell type (B cell, T cell or NK cell) and rate of progression (aggressive or indolent). The percentages listed reflect the frequency of diagnosed cases of the most common NHL subtypes. 

Mature B-cell lymphomas (about 85%-90% of NHL cases)

Aggressive

  • Diffuse large B-cell lymphoma (DLBCL) (30%)
  • Mantle cell lymphoma (MCL) (3%)—has features of both indolent and aggressive NHL
  • Lymphoblastic lymphoma (2%)
  • Burkitt lymphoma (BL) (2%)
  • Primary mediastinal (thymic) large B-cell lymphoma (PMBCL)
  • Transformed follicular and transformed mucosa-associated lymphoid tissue (MALT) lymphomas
  • High-grade B-cell lymphoma with double or triple hits (HBL)
  • Primary cutaneous DLBCL, leg type
  • Primary DLBCL of the central nervous system
  • Primary central nervous system (CNS) lymphoma
  • Acquired immunodeficiency syndrome (AIDS)-associated lymphoma

Indolent

  • Follicular lymphoma (FL) (22%)
  • Marginal zone lymphoma (MZL) (7%)
  • Chronic lymphocytic leukemia/small-cell lymphocytic lymphoma (CLL/SLL) (7%)
  • Gastric mucosa-associated lymphoid tissue (MALT) lymphoma (8%)
  • Lymphoplasmacytic lymphoma (1%)
  • Waldenström macroglobulinemia (WM)
  • Nodal marginal zone lymphoma (NMZL) (1%)
  • Splenic marginal zone lymphoma (SMZL)

Mature T-cell and natural killer (NK)-cell lymphomas (about 10%-15% of NHL cases)

Systemic

  • Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (6%)
  • Systemic anaplastic large-cell lymphoma (ALCL) (2%)
  • Lymphoblastic lymphoma (2%)
  • Hepatosplenic T-cell lymphoma
  • Enteropathy-associated intestinal T-cell lymphoma
    • Monomorphic epitheliotropic intestinal T-cell lymphoma
  • Angioimmunoblastic T-cell lymphoma (AITL)
  • Adult T-cell leukemia/lymphoma
  • Extranodal natural killer (NK)/T-cell lymphoma (ENK/TCL), nasal type

Primary cutaneous

  • Cutaneous T-cell lymphoma (CTCL) (4%)
    • Mycosis fungoides (MF)
    • Sézary syndrome (SS)
  • Primary cutaneous anaplastic large-cell lymphoma (pcALCL)
  • Subcutaneous panniculitis-like T-cell lymphoma (SPTCL)
    • Primary cutaneous gamma delta T-cell lymphoma

Source: This table is based on information presented in The 2016 revision of the World Health Organization classification of lymphoid neoplasms. The descriptive parts of the names (eg, follicular, mantle cell or marginal zone) of some disease subtypes refer to the specific areas of normal lymph nodes where the lymphoma originated.

 

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