How is Diffuse Large B Cell Lymphoma Staged and Classified?

Diffuse large B-cell lymphoma (DLBCL) is staged using the Ann Arbor staging system, which is commonly used for all types of lymphoma. This system takes into account the number of lymph node regions involved, whether these regions are on one or both sides of the diaphragm, and whether there is extranodal disease. The stages are as follows:

• Stage I: The cancer is in a single lymph node region or a single organ.
• Stage II: The cancer is in two or more lymph node regions on the same side of the diaphragm, or the cancer extends from a single lymph node region into a nearby organ.
• Stage III: The cancer is in lymph node regions on both sides of the diaphragm.
• Stage IV: The cancer has spread to one or more organs outside the lymphatic system.
• X: Bulk larger than 10cm 
• E: Extension outside of the lymph nodes or only one isolated site of outside the lymph nodes
• A/B: B symptoms: weight loss > 10%, fever, drenching night sweats

Each stage can also be classified as "A" or "B" depending on whether the patient has specific symptoms. "A" means the patient does not have fever, night sweats, or weight loss. "B" means the patient has at least one of these symptoms.

How is Diffuse Large B Cell Lymphoma Classified? 

DLBCL is classified based on its cell of origin and genetic alterations. The two main subtypes determined through immunohistochemistry or gene expression profiling are:

• Germinal Center B-cell-like (GCB): This subtype generally has a better prognosis.
• Activated B-cell-like (ABC): This subtype often has a poorer prognosis.

DLBCL can also be classified based on genetic alterations. For example, DLBCL with MYC and BCL2 and/or BCL6 rearrangements is known as "double-hit" or "triple-hit" lymphoma and is associated with a poor prognosis.

What Other Classifications Exist for DLBCL?

The World Health Organization (WHO) further classifies DLBCL into different forms, including:

• DLBCL, not otherwise specified (NOS):
  • Germinal center B-cell type
  • Activated B-cell type
• Other forms:
  • Primary DLBCL of the central nervous system (CNS)
  • Primary cutaneous DLBCL, leg type
  • Epstein-Barr virus (EBV)–positive DLBCL, NOS
  • DLBCL associated with chronic inflammation
  • Human herpes virus 8 (HHV8)–positive DLBCL, NOS

What are the Phases of Diffuse Large B Cell Lymphoma?

The phases of DLBCL can be understood in terms of how it progresses and how it responds to treatment.

• Diagnostic Phase: Symptoms are identified, and tests are conducted to confirm the diagnosis.
• Staging Phase: The extent of the disease is determined.
• Treatment Phase: Treatment options, including chemotherapy, radiation therapy, or stem cell transplant, are chosen based on the disease stage, patient’s health, and DLBCL subtype.
• Remission Phase: If treatment is successful, the disease is not active. This phase can last for many years, but DLBCL can relapse in about half of the patients.
• Relapse Phase: The disease becomes active again, requiring different treatment regimens, targeted therapies, or another stem cell transplant to achieve remission.

What is the DLBCL Risk Stratification? 

The IPI score is used to plan better treatment options to prevent the DLBCL to come back after treatment

Factors (1 point for each factor present):

• Age ≥60y
• Eastern Cooperative Oncology Group (ECOG) performance status ≥2
• Elevated lactate dehydrogenase (LDH) level
• Ann Arbor stage III or IV
• ≥2 extranodal sites of disease

Risk category (factors):

• Low (0 or 1)
• Low-intermediate (2)
• High-intermediate (3)
• High (4 or 5)

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Source: 

• Diffuse Large B-Cell Lymphoma (Non-Hodgkin Lymphoma) Staging