What are the characteristics of Non-Hodgkin Lymphoma (NHL)?

From the Guidelines

Non-Hodgkin lymphoma (NHL) is a heterogeneous group of lymphoproliferative disorders originating in B lymphocytes, T lymphocytes, or natural killer cells, characterized by abnormal growth of lymphocytes in lymph nodes, spleen, bone marrow, or other organs. The key characteristics of NHL include a diverse range of presentations and behaviors, with some types being aggressive (fast-growing) like diffuse large B-cell lymphoma, and others being indolent (slow-growing) like follicular lymphoma 1. NHL typically presents with painless, enlarged lymph nodes, though patients may also experience systemic B symptoms including fever, night sweats, and unintentional weight loss, as well as fatigue, recurrent infections, and easy bruising due to bone marrow involvement 1.

Classification and Risk Factors

NHL is classified into different types based on cell origin (B-cell or T-cell), with B-cell lymphomas accounting for about 85% of cases 1. Risk factors for NHL include advanced age, immune system disorders, certain infections like HIV or Epstein-Barr virus, and exposure to certain chemicals 1.

Diagnosis and Treatment

Diagnosis of NHL requires a lymph node biopsy with immunohistochemistry, flow cytometry, and sometimes genetic testing to determine the specific subtype, which guides treatment decisions 1. Treatment options for NHL vary depending on the specific subtype and stage of the disease, but may include rituximab-based chemoimmunotherapy, high-dose therapy and autologous stem cell rescue (HDT/ASCR), and targeted therapies like ibrutinib 1.

Regional Specificities

It's worth noting that there are regional specificities in the incidence of lymphomas, with a significantly lower incidence of follicular lymphoma and chronic lymphocytic leukemia, and a significantly higher incidence of T-cell lymphomas in Asian countries compared with Europe and North America 1. Additionally, some Middle East countries have a high incidence of Epstein-Barr virus–associated lymphomas, and HTLV-1–associated ATL is endemic in some Middle East regions 1.

Management

Management of malignant lymphomas in developing countries is variable and largely depends on the availability of diagnostic and therapeutic resources, such as immunohistochemistry and molecular techniques, PET-CT scan, and expensive targeted therapies 1. Therefore, it is essential to consider the specific subtype, stage, and regional specificities when making treatment decisions for patients with NHL.

From the FDA Drug Label

Patients with previously untreated diffuse large B-cell NHL received RITUXAN in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or other anthracycline-based chemotherapy regimens Among all enrolled patients, 62% had centrally confirmed DLBCL histology, 73% had Stage III–IV disease, 56% had IPI scores greater than or equal to 2,86% had ECOG performance status of < 2,57% had elevated LDH levels, and 30% had two or more extranodal disease sites involved Thirty-seven percent of the study population was greater than 60 years of age, 99% had Stage III or IV disease, and 63% had an IPI score greater than or equal to 2. Advanced stage is defined as Stage III with elevated lactose dehydrogenase (LDH) level [LDH greater than twice the institutional upper limit of the adult normal values] or stage IV B-cell NHL or B-AL Disease Type B-AL37%34% Burkitt or Burkitt-like NHL55%56% DLBCL8%8% Bone marrow involvement45%45% CNS involvement27%27%

The characteristics of Non-Hodgkin Lymphoma (NHL) include:

• Advanced stage: Stage III with elevated lactose dehydrogenase (LDH) level or stage IV B-cell NHL or B-AL
• Age: 37% of patients were greater than 60 years of age
• Disease type:
  • B-AL: 37%
  • Burkitt or Burkitt-like NHL: 55%
  • DLBCL: 8%
• Stage:
  • Stage III: with elevated LDH level
  • Stage IV: B-cell NHL or B-AL
• IPI score: 63% had an IPI score greater than or equal to 2
• ECOG performance status: 86% had ECOG performance status of < 2
• LDH levels: 57% had elevated LDH levels
• Extranodal disease sites: 30% had two or more extranodal disease sites involved
• Bone marrow involvement: 45%
• CNS involvement: 27% 2

From the Research

Characteristics of Non-Hodgkin Lymphoma (NHL)

• Non-Hodgkin lymphomas (NHLs) are a heterogeneous group of lymphoid neoplasms with different biological characteristics 3, 4.
• About 90% of all lymphomas in the United States originate from B lymphocytes, while the remaining originate from T cells 3, 4, 5.
• NHL is a diverse collection of malignant neoplasms with lymphoid-cell origin, which includes all the malignant lymphomas that are not classified as Hodgkin lymphoma 6.
• The prognosis of a newly diagnosed NHL patient depends on the specific subtype of lymphoma, stage of the disease, and age of the patient 6.

Staging of Non-Hodgkin Lymphoma

• The American Cancer Society describes the staging of lymphoma as follows:
  • Stage I is lymphoma in a single node or area.
  • Stage II is when that lymphoma has spread to another node or organ tissue.
  • Stage III is when it has spread to lymph nodes on two sides of the diaphragm.
  • Stage IV is when cancer has significantly spread to organs outside the lymph system 3, 4.
• The Ann Arbor Staging Classification is used routinely to classify the extent of disease 5.

Treatment of Non-Hodgkin Lymphoma

• The treatment of NHLs depends on the neoplastic histology and stage of the tumor, which will indicate whether radiotherapy, chemotherapy, or a combination is the best suitable treatment 3, 4.
• Radiation therapy is the traditional therapeutic route for localized follicular and mucosa-associated lymphomas 3, 4.
• Chemotherapy is utilized for the treatment of large-cell lymphomas and high-grade lymphomas 3, 4.
• Immunotherapy and radioimmunotherapy are also used in the treatment of NHL 5.