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Navigating the Different Types of Non-Hodgkin Lymphoma

Posted on March 24, 2022
Medically reviewed by
Mark Levin, M.D.
Article written by
Aminah Wali, Ph.D.

Lymphoma is broadly categorized into two groups: Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). NHL is more common and accounts for about 4 percent of all cancer diagnoses in the United States. It has been estimated that there are more than 80,000 new cases of NHL each year in the U.S. Although NHL can be diagnosed at any age, most forms of the disease primarily affect older adults.

NHL affects cells of the immune system. Cancer can form in different types of white blood cells (lymphocytes), which have various roles in helping the body fight against infection.

There are many different subtypes of NHL. They can be classified as either aggressive (fast-growing or high-grade) or indolent (slow-growing or low-grade) cancers. NHL cancer cells accumulate in tissues of the lymphatic system. This is most commonly the lymph nodes and bone marrow, but also includes the spleen, thymus, and gastrointestinal tract.

B-Cell Lymphoma

The most common type of NHL is B-cell lymphoma, which forms from B lymphocytes or B cells. B-cell lymphoma makes up about 85 percent of NHL and includes several different subtypes.

Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of NHL, accounting for about a third of all cases. It forms as a mass in the lymph nodes, but can also originate in other parts of the body. Based on where it forms, DLBCL might be further grouped into smaller subtypes:

Although DLBCL is an aggressive (fast-growing) type of cancer, when caught early enough, it generally responds to treatment with intensive chemotherapy regimens administered in combination with the antibody drug rituximab (Rituxan). Radiation therapy may also be used, particularly for early-stage disease.

Read more about diffuse large B-cell lymphoma.

Burkitt Lymphoma

Burkitt lymphoma is an aggressive B-cell lymphoma that is more commonly diagnosed in children and young adults. It is associated with Epstein-Barr virus infection and HIV infection. Burkitt lymphoma requires treatment with an intensive chemotherapy regimen and, if caught early enough in children, has a high survival rate. Adults diagnosed with Burkitt lymphoma tend to have harder-to-treat disease and a poorer prognosis.

Read more about Burkitt lymphoma.

Double-Hit Lymphoma and Triple-Hit Lymphoma

Double-hit lymphoma and triple-hit lymphoma are rare types of cancer that contain genetic rearrangements in either two or three genes, respectively. Double-hit lymphoma and triple-hit lymphoma are very aggressive diseases that don’t respond well to conventional treatments. People with these types of lymphoma have a poor prognosis.

Read more about double-hit lymphoma.

Follicular Lymphoma

Follicular lymphoma gets its name from its ability to form in circular structures called follicles. It is the most common indolent (slow-growing) form of NHL. Although it is generally not curable, because it grows slowly, it is generally managed by close observation. Most people live with this type of cancer for years before treatment is required.

Read more about follicular lymphoma.

Mantle Cell Lymphoma

Mantle cell lymphoma is a rare, aggressive disease that often forms in the gastrointestinal tract and bone marrow. It is usually not diagnosed until it is in the late stages and consequently, those with this type of lymphoma have a rather poor prognosis. The primary treatment for mantle cell lymphoma is chemotherapy, and a stem cell transplant may be recommended to achieve higher doses of chemotherapy.

Read more about mantle cell lymphoma.

Marginal Zone Lymphoma

Marginal zone lymphoma (MZL) is an indolent form of NHL that arises from B cells from the marginal zone of the spleen. MZL can be further categorized into three distinct subtypes, depending on where cancer forms.

Read more about marginal zone lymphoma.

Mucosa-Associated Lymphoid Tissue Lymphoma

Mucosa-associated lymphoid tissue lymphoma, also called extranodal marginal zone B-cell lymphoma or MALT lymphoma, is the most common form of MZL. It occurs outside of the lymph nodes, usually in the stomach, and is often associated with infection of the bacterium Helicobacter pylori. Mucosa-associated lymphoid tissue lymphoma is usually treated with antibiotics and people with this disease generally have a favorable prognosis.

Read more about MALT lymphoma.

Nodal Marginal Zone Lymphoma

Nodal marginal zone B-cell lymphoma occurs in the lymph nodes and bone marrow. It is the rarest form of MZL and may not require treatment.

Splenic Marginal Zone Lymphoma

Splenic marginal zone B-cell lymphoma forms in the spleen. Like nodal MZL, it does not always require treatment.

Read more about splenic marginal zone lymphoma.

Waldenströms Macroglobulinemia

Also known as lymphoplasmacytic lymphoma, Waldenström’s macroglobulinemia is a very rare NHL characterized by small lymphocytes. Waldenström’s macroglobulinemia is a generally slow-growing cancer that affects older adults. Due to its rarity and lack of defining features, it can only be diagnosed after ruling out other more common B-cell lymphomas.

Read more about Waldenström’s macroglobulinemia.

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) are two very closely related types of cancer. Both diseases have the same cancer cells originating in B cells, but SLL forms in the spleen and lymph nodes while CLL forms primarily in the bone marrow. CLL/SLL is an indolent form of NHL that may not cause many symptoms and is often managed initially by surveillance before treatment is required.

Read more about chronic lymphocytic leukemia and small lymphocytic lymphoma.

Cutaneous B-Cell Lymphoma

Primary cutaneous B-cell lymphoma (CBCL) forms as skin lesions. These make up approximately 5 percent of all NHLs. CBCL has several different subtypes:

  • Primary cutaneous follicle center lymphoma — An indolent disease that forms a bumpy rash on the head, neck, and torso
  • Primary cutaneous marginal zone B-cell lymphoma — An indolent disease that forms red skin lesions on the torso and arms
  • Primary cutaneous diffuse large B-cell lymphoma, leg type — A more aggressive cancer that presents as tumor nodules on the legs, arms, and torso
  • Primary cutaneous diffuse large B-cell lymphoma, other — A very rare, aggressive disease that forms on the torso, head, and limbs

Although the indolent forms of CBCL may not require treatment and are commonly managed by active surveillance (the watch-and-wait approach), aggressive CBCLs are generally treated with chemotherapy plus rituximab.

Read more about cutaneous B-cell lymphoma.

Hairy Cell Leukemia

Hairy cell leukemia is categorized as leukemia and may also be classified as a type of B-cell lymphoma. It is a slow-growing type of cancer that forms in the spleen and is more common in older adults, particularly in men.

T-Cell Lymphoma

The less common type of NHL is T-cell lymphoma. Overall, T-cell lymphoma is much more rare than B-cell lymphoma. T-cell lymphoma makes up about 15 percent of NHL and also contains several subtypes.

T-Cell Lymphoblastic Lymphoma/Leukemia

T-cell lymphoblastic lymphoma accounts for about 20 percent of NHL cases in children. It is also considered a form of leukemia if there is a significant number of cancer cells in the bone marrow. It is an aggressive form of NHL, but people generally respond to chemotherapy. However, when T-cell lymphoblastic lymphoma/leukemia has spread to bone marrow or has come back (relapsed) after initially responding to treatment, it is much more difficult to treat.

Peripheral T-Cell Lymphoma

Peripheral T-cell lymphoma (PTCL) is a group of rare cancers that form in tissues outside of the bone marrow. Overall, it is more common in Asia and the Caribbean than in the United States. PTCL is primarily an aggressive cancer that contains several subtypes.

Peripheral T-Cell Lymphoma, Not Otherwise Specified

The largest subtype of PTCL is peripheral T-cell lymphoma, not otherwise specified, which includes all “generic” forms of PTCL that don’t fit into one of the more specific subtypes. Due to the heterogeneity of this subtype, the presentation of the disease can vary from person to person. Although chemotherapy is the typical treatment approach, there is no standard treatment regimen for peripheral T-cell lymphoma, not otherwise specified. The overall prognosis remains poor.

Cutaneous T-Cell Lymphoma

Cutaneous T-cell lymphoma (CTCL) is a more common type of T-cell lymphoma that primarily forms a skin rash but can also be found in the blood and lymph nodes. There are multiple types of CTCL, including mycosis fungoides and Sézary syndrome. CTCL is often treated with skin-directed therapies.

Read more about cutaneous T-cell lymphoma.

Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma forms primarily in the liver and spleen. It is an extremely rare disease with fewer than 30 cases diagnosed per year and is most commonly diagnosed in younger adult men. Due to a lack of effective treatment options, people diagnosed with hepatosplenic T-cell lymphoma are often encouraged to participate in clinical trials.

Adult T-Cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is a rare T-cell malignancy that can be classified as either leukemia (if the cancer forms in the blood) or lymphoma (if it forms in the lymph node). ATLL is caused by human T-cell lymphotropic virus type 1 infection. There are four subtypes of ATLL:

  • Acute — An aggressive disease with cancer cells in the blood
  • Lymphomatous — An aggressive disease with cancer cells in the lymph nodes
  • Chronic — Low-grade cancer that may cause an itchy rash
  • Smoldering — Another low-grade disease that may not cause any symptoms

Aggressive forms of the disease are targeted with chemotherapy, but for slow-growing cancers, doctors may opt to postpone treatment and just keep an eye on disease progression.

Read more about adult T-cell leukemia/lymphoma.

Angioimmunoblastic T-Cell Lymphoma

Angioimmunoblastic T-cell lymphoma, while rare overall, is one of the more common subtypes of T-cell lymphoma, accounting for about two percent of NHL cases in the United States. It is most commonly diagnosed in older adults at the later stages of the disease. A person diagnosed with angioimmunoblastic T-cell lymphoma may be prescribed steroids to reduce inflammation caused by cancer and is typically treated with chemotherapy.

Read more about angioimmunoblastic T-cell lymphoma.

Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma (ALCL) makes up about 1 percent of NHL cases and is diagnosed in children and adults. There are different forms of ALCL:

  • People with primary cutaneous ALCL present with skin lesions.
  • Systemic ALCL forms in the lymph nodes and elsewhere throughout the body. There are two types of this disease — ALK-positive ALCL (contains a mutation in a gene called ALK) or ALK-negative ALCL.
  • Breast implant-associated ALCL can occur at the site of a breast implant, but this is very rare.

Radiation is often used as a treatment for people with primary cutaneous ALCL. For those with systemic ALCL, the ALK-positive subset responds well to chemotherapy treatment. People with ALK-negative ALCL do not respond as well to treatment and have a poorer prognosis.

Read more about anaplastic large cell lymphoma.

Enteropathy-Associated Intestinal T-Cell Lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a type of cancer that forms in the lining of the small intestine. It is associated with an autoimmune condition known as celiac disease. Although treatment with chemotherapy is possible, a person with EATL may be encouraged to take part in a clinical trial.

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma

Previously considered a subset of EATL, monomorphic epitheliotropic intestinal T-cell lymphoma is another type of lymphoma that occurs in the intestines but is not linked to celiac disease. It is much rarer than EATL and has a similar treatment strategy.

Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

Extranodal natural killer/T-cell lymphoma, nasal type is a type of cancer that can form from two different types of white blood cells, T cells and natural killer cells. This cancer forms in the nose and is associated with Epstein-Barr virus infection. Treatment often includes radiation to shrink the nasal tumor, as well as chemotherapy.

Talk With Others Who Understand

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Have you or a loved one been diagnosed with non-Hodgkin lymphoma? Share your experience in the comments below, or start a conversation by posting on your Activities page.

References
  1. What Is Non-Hodgkin Lymphoma? — American Cancer Society
  2. NHL Subtypes — Leukemia & Lymphoma Society
  3. Types of B-Cell Lymphoma — American Cancer Society
  4. Double Hit Lymphoma (DHL) Triple Hit Lymphoma (THL) — Atlas of Genetics and Cytogenetics in Oncology and Haematology
  5. High-Grade B-Cell Lymphoma With Rearrangements of MYC and BCL2 and/or BCL6: Double Hit and Triple Hit Lymphomas and Double Expressing Lymphoma — Blood Reviews
  6. Mantle Cell Lymphoma — Lymphoma Research Foundation
  7. Mantle Cell Lymphoma: 2017 Update on Diagnosis, Risk‐Stratification, and Clinical Management — American Journal of Hematology
  8. High-Dose Chemotherapy and Stem Cell Transplant for Non-Hodgkin Lymphoma — American Cancer Society
  9. Marginal Zone Lymphoma — Lymphoma Research Foundation
  10. Marginal Zone B-Cells, a Gatekeeper of Innate Immunity — Frontiers in Immunology
  11. MALT Lymphoma: Epidemiology, Clinical Diagnosis and Treatment — Journal of Medicine and Life
  12. Gastric Mucosa-Associated Lymphoid Tissue Lymphoma and Helicobacter Pylori Infection: A Review of Current Diagnosis and Management — Biomarker Research
  13. Marginal Zone Lymphoma: Treatment Options — Lymphoma Research Foundation
  14. What Is Waldenstrom Macroglobulinemia? — American Cancer Society
  15. Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma — Lymphoma Research Foundation
  16. Cutaneous B-Cell Lymphoma — Lymphoma Research Foundation
  17. Hairy Cell Leukemia: Present and Future Directions — Leukemia & Lymphoma
  18. High-Dose Chemotherapy and Stem Cell Transplant for Non-Hodgkin Lymphoma — American Cancer Society
  19. About Childhood Primary Mediastinal B-Cell Lymphoma (PMBL) — Dana-Farber Cancer Institute
  20. Types of T-Cell Lymphoma — American Cancer Society
  21. T-Cell Lymphoblastic Lymphoma and Leukemia: Different Diseases From a Common Premalignant Progenitor? — Blood Advances
  22. Peripheral T-Cell Lymphoma — Lymphoma Research Foundation
  23. Peripheral T-Cell Lymphoma, Not Otherwise Specified — T-Cell Leukemia Lymphoma Foundation
  24. Peripheral T Cell Lymphoma, Not Otherwise Specified (PTCL‐NOS). A New Prognostic Model Developed by the International T Cell Project Network — British Journal of Haematology
  25. Cutaneous T-Cell Lymphoma — Lymphoma Research Foundation
  26. Hepatosplenic T-Cell Lymphoma (HSTCL) — T-Cell Leukemia Lymphoma Foundation
  27. Incidence, Clinical Findings, and Survival of Hepatosplenic T-Cell Lymphoma in the United States — American Journal of Hematology
  28. Angioimmunoblastic T-Cell Lymphoma — Lymphoma Research Foundation
  29. Anaplastic Large Cell Lymphoma — Lymphoma Research Foundation
  30. Anaplastic Large Cell Lymphoma: Treatment Options — Lymphoma Research Foundation
  31. Intestinal T-Cell Lymphomas — Lymphoma Action
  32. Extranodal NK/T-Cell Lymphoma, Nasal Type — Lymphoma Action
All updates must be accompanied by text or a picture.
Mark Levin, M.D. is a hematology and oncology specialist with over 37 years of experience in internal medicine. Review provided by VeriMed Healthcare Network. Learn more about him here.
Aminah Wali, Ph.D. received her doctorate in genetics and molecular biology from the University of North Carolina at Chapel Hill. Learn more about her here.

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