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Diffuse Large B-Cell Lymphomas: NCCN Guidelines for Patients

July 29, 2022 - read ≈ 5 min

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The NCCN Guidelines for Patients were developed by the National Comprehensive Cancer Network (NCCN). NCCN is an organization that unites leading cancer care centers in the United States of America. Its activities are focused on scientific research, treatment and education of patients.

Lymphoma basics

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). NHLs start in lymphocytes, a type of white blood cell. DLBCL is a fast-growing cancer, affecting tissues and organs such as bone marrow, spleen, thymus, lymph nodes, lymphatic vessels, and other parts of the body.

Lymphatic system

The lymphatic or lymph system is a major part of the body’s immune system. It is a germ-fighting network of tissues and organs that includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels.

Lymphatic vessels are a network of thin tubes that carry lymphatic fluid (lymph) and white blood cells into all the tissues of the body. Lymph gives cells water and food. White blood cells help fight infection and disease.

As lymph travels throughout your body, it passes through hundreds of small bean-shaped structures called lymph nodes. Lymph nodes make immune cells that help the body fight infection. They also filter the lymph fluid and remove foreign material such as bacteria and cancer cells.

Lymphocytes

Non-Hodgkin lymphoma (NHL) is a cancer of the immune system that starts in lymphocytes. A lymphocyte is a type of immune cell that is also a type of white blood cell. White blood cells fight infection. Lymphocytes are found

in both blood and lymph tissue. Lymph tissue includes lymph vessels and lymph nodes. Lymphocytes normally grow in response to infection or inflammation. When they grow on their own without proper regulation, they can develop into a lymphoma.

There are 3 main types of lymphocytes:

  • B lymphocytes or B cells make antibodies. An antibody is a protein.
  • T lymphocytes or T cells help kill tumor cells and infected cells directly in a targeted and specific way. T cells help control immune responses and form specific immune memory.
  • Natural killer (NK) cells can kill tumor cells or virus-infected cells.

NHL can be formed from either B-cell, T-cell, or NK-cell lymphocytes. Diffuse large B-cell lymphoma starts in mature B cells. B cells produce antibodies that are used to attack invading bacteria, viruses, and toxins. The antibody molecules latch onto and destroy invading viruses or bacteria.

DLBCL

Diffuse large B-cell lymphoma (DLBCL) is the most common type of NHL. It accounts for about 3 out of every 10 NHLs. Large-celled, fast-growing tumors are commonly found in lymph nodes, spleen, liver, bone marrow,

or other tissues and organs. Symptoms can include fever, night sweats, fatigue, and weight loss. These symptoms are referred to as B symptoms. Not everyone has the same symptoms and tumors can be found anywhere in the body.

DLBCL subtypes

There are many subtypes of DLBCL. If you have DLBCL along with another lymphoma, it will likely be treated as DLBCL. For DLBCL subtypes covered in this book, see Guide 1.

I had none of the typical DLBCL symptoms. Nothing about my experience was typical. I was young, in my 20s, and had DLBCL in a bone in my foot.


Guide 1 DLBCL subtypes

  • DLBCL, not otherwise specified (NOS) (includes germinal center and nongerminal center)
    • Germinal center (or follicle center) is not the same as follicular lymphoma (FL) and can occur in DLBCL and Burkitt lymphoma.
  • DLBCL with follicular lymphoma (FL) of any grade
  • DLBCL with gastric mucosa-associated lymphoid tissue (MALT) lymphoma
  • DLBCL with nongastric MALT lymphoma
  • Intravascular large B-cell lymphoma
  • DLBCL associated with chronic inflammation
  • ALK-positive large B-cell lymphoma
  • Epstein-Barr virus-positive (EBV-positive) DLBCL, NOS
  • T-cell/histiocyte-rich large B-cell lymphoma
  • Fibrin-associated DLBCL (FA-DLBCL)
  • Large B-cell lymphoma with IRF4 rearrangement
  • Double-expressor DLBCL
  • Primary mediastinal large B-cell lymphoma (PMBL)
  • Gray zone lymphoma (also known as B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Hodgkin lymphoma)
  • High-grade B-cell lymphomas with translocations of MYC and BCL2 and/or BCL6 (double- or triple-hit lymphoma)
  • High-grade B-cell lymphomas, NOS
  • Primary cutaneous DLBCL, leg type

Information on subtypes not covered in this book:

  • Primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL) – For more information, read the NCCN Guidelines for Patients: Primary Cutaneous Lymphomas and NCCN Guidelines for Patients: B-Cell Lymphomas – Follicular Lymphoma, available at NCCN.org/patientguidelines.
  • Primary DLBCL of the central nervous system (CNS) – For more information, read the NCCN Guidelines for Patients: Central Nervous System Cancers – Primary Central Nervous System Lymphoma, available at NCCN.org/patientguidelines.
  • DLBCL arising from chronic lymphocytic leukemia (CLL) called Richter’s transformation – For more information, read the NCCN Guidelines for Patients: Chronic Lymphocytic Leukemia, available at NCCN.org/patientguidelines.

Those with diffuse large B-cell lymphoma or any of its subtypes should be treated at centers experienced in your type of cancer.

Key points

  • The lymphatic or lymph system is a network of tissues and organs that helps your body fight infections and disease. It is part of the immune system.
  • Non-Hodgkin lymphomas (NHLs) start in lymphocytes, a type of immune and white blood cell. White blood cells fight infection.
  • Diffuse large B-cell lymphoma (DLBCL) is the most common type of NHL.
  • Large-celled, fast-growing tumors are commonly found in lymph nodes, spleen, liver, bone marrow, or other tissues and organs. Symptoms include fever, night sweats, and weight loss.
  • There are many subtypes of DLBCL.


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