Non Hodgkin's Lymphoma Types - Common & Rare Types

Non-Hodgkin’s lymphoma (NHL) is a type of cancer originating in the lymphatic system, a vital part of the body’s immune system. It involves the abnormal growth of B lymphocytes (B-cells) or T lymphocytes (T-cells). These white blood cells can accumulate and form tumours in various lymph nodes or other lymphatic tissues.

According to the World Health Organisation (WHO), more than 60 specific non-Hodgkin’s lymphoma types exist. These subtypes are categorised by the characteristics of the lymphoma cells.

While NHL comprises numerous variations, several types are more commonly diagnosed and studied. Accurate diagnosis and proper classification are essential for determining the most suitable treatment approach and achieving favourable outcomes. 

Some of the common non-Hodgkin’s lymphoma type B cancers include:

1. Diffuse Large B-cell Lymphoma (DLBCL): It is the most prevalent type of NHL, accounting for a significant portion of cases. According to NCBI, DLBCL accounts for 25% to 30% of all NHL cases.

   It is an aggressive lymphoma that can develop in lymph nodes or extranodal sites. Non-Hodgkin’s diffuse large cell type B lymphoma often grows rapidly and requires prompt treatment.

2. Follicular Lymphoma: This is the second most common form of non-Hodgkin’s lymphoma, accounting for about 22% of all cases.
   It is a slow-growing form of NHL that often develops in the lymph nodes, bone marrow, or other organs. 

   Follicular lymphoma is usually not curable. However, treatment can improve a person’s quality of life and help with prolonged survival. 

T-cell lymphomas are usually less common than B-cell lymphoma. However, within T-cell lymphoma, the most common type includes:

1. Peripheral T-cell Lymphoma, Not Otherwise Specified: These lymphomas represent a group of aggressive cancers that begin in T-cells.

   The most common is called “not otherwise specified” because it does not fit into more specific classifications.

   According to an NCBI study, the incidence of peripheral T-cell lymphoma is about 20% in India. It can involve the lymph nodes, liver, bone marrow, intestines, and skin.  

Rare Types of Non-Hodgkin’s Lymphoma

Certain subtypes are notably rare among the wide range of non-Hodgkin’s lymphomas. These rare types of NHL often pose diagnostic challenges. 

Examples of uncommon non-Hodgkin’s lymphoma type B cancers include:

1. Chronic Lymphocytic Leukaemia (CLL)/ Small Lymphocytic Lymphoma (SLL): These conditions are essentially the same, characterised by abnormal B-cell growth. CLL mainly affects the blood, while SLL primarily involves lymph nodes.

   While CLL is the most common adult leukaemia in the United States, the disease is uncommon in India.

2. Mantle Cell Lymphoma: This rare subtype of NHL mostly affects men aged 60 and older. NCBI states that mantle cell lymphoma comprises about 5% of all NHL cases. It grows in the mantle zone, which is the outer ring of the small lymphocytes.

   This type is usually found in the lymph nodes, bone marrow, and spleen. 

3. Marginal Zone Lymphoma: This subtype originating in the marginal zone of lymphoid tissues accounts for 3-9% of NHL cases.8 The marginal zone is the area surrounding the mantle zone.

   This type of lymphoma can affect various sites, such as the skin, lungs, stomach, or salivary glands. It includes three main variants: extranodal, nodal, and splenic marginal zone lymphoma.

4. Burkitt Lymphoma: It is an aggressive NHL subtype, often presenting with rapidly growing tumours.

   Burkitt lymphoma is usually found in the stomach and is more common in children than adults. It makes up about 2% of all lymphomas.

5. Lymphoplasmacytic Lymphoma: This is a rare (1%-2%), slow-growing cancer that forms in the liver and spleen.

   It produces an antibody called M-protein that may cause specific signs and symptoms. 

Rare types of T-cell non-Hodgkin’s lymphoma include:

1. Cutaneous T-cell Lymphoma: A cancer in which the T-cells attack the skin. It accounts for about 4% of NHL cases. Examples of cutaneous T-cell lymphoma include Mycosis fungoides and Sezary syndrome. 

2. Adult T-cell Lymphoma/Leukaemia: ATLL is caused by the human T-cell lymphotropic virus type 1 (HTLV-1). It may occur in bones, skin, and blood.

   ASCO Journal says the risk of developing ATLL is about 4%-7% in HTLV-1 carriers.

3. Anaplastic Large Cell Lymphoma (ALCL): This fast-growing lymphoma accounts for about 2% of lymphomas. They are divided into two subtypes: primary cutaneous ALCL (only affects the skin) and systemic ALCL (affects lymph nodes and other organs). 

4. Angioimmunoblastic T-cell Lymphoma: Constituting about 4% of all lymphomas, this lymphoma spreads quickly and often returns after treatment.

   It generally presents with enlarged lymph nodes, skin rashes, weight loss, fever, and infections. 

The symptoms of non-Hodgkin’s lymphoma can vary depending on its subtype, stage, and affected organs. Symptoms of NHL are non-specific and may be similar to those of other diseases.

While some symptoms may appear early, others may be experienced when the disease progresses.

The most common early indicator of NHL include:

1. Swollen lymph nodes: Painless, enlarged lymph nodes, often in the neck, armpits, or groyne, is usually the first warning sign of NHL. It may also be the only sign until the disease progresses. 

Advanced Symptoms of NHL include:

1. Cough, Chest Pain, or Shortness of Breath: When NHL affects the chest, it can cause persistent coughing, chest pain, or shortness of breath.

2. Fatigue: Profound tiredness or weakness, unrelated to activity, is a common symptom.

3. Abdominal Pain: Swelling, pain, or discomfort in the abdomen can indicate NHL in the abdominal area.

4. Itching or Skin Changes: Persistent itching, rashes, or changes in skin appearance may occur due to NHL. 

5. Early Satiety: Individuals may experience a feeling of fullness quickly after eating only a small amount of food. This happens when enlarged lymph nodes or tumours compress the stomach or other nearby organs. 

6. Easy Bruising or Bleeding: Low platelet count might result in easy bruising, bleeding gums, or nosebleeds.

7. Frequent Infections: Weakened immune responses can make individuals more susceptible to infections.

Accurate NHL diagnosis is essential for tailoring effective treatment strategies and improving outcomes. The diagnosis of NHL is a comprehensive process involving various steps:

1. Medical History and Physical Exam: A doctor gathers information about symptoms, medical history, and risk factors. This is followed by a thorough physical examination to assess swollen lymph nodes, spleen, or liver.

2. Imaging Studies: Tests like CT scans, PET scans, and MRIs help visualise lymph nodes, organs, and potential tumour masses. This aids in staging and determining the extent of the disease.

3. Blood and Urine Tests: These tests are performed to rule out infections or other diseases. 

4. Lymph Node Biopsy: The doctor removes all or part of the lymph nodes for laboratory testing. 

5. Bone Marrow Biopsy: In some cases, a bone marrow biopsy is performed to assess the presence of lymphoma cells in the bone marrow. 

6. Lumbar Puncture (Spinal Tap): The test may be performed for lymphomas involving the central nervous system. It helps analyse cerebrospinal fluid.

Treatment depends on the non-Hodgkin’s lymphoma types, stage, patient’s health, and other factors. The goal is to achieve remission, alleviate symptoms, and improve quality of life. Treatments for NHL include:

1. Watchful Waiting: No immediate treatment is initiated in certain low-grade or indolent lymphomas. The patient is closely monitored until treatment becomes necessary.

2. Chemotherapy: It involves the use of powerful drugs to kill or control cancer cells. Combination chemotherapy regimens are often employed. These are usually given intravenously.

3. Targeted Therapies: These drugs specifically target certain molecules involved in lymphoma cell growth. Monoclonal antibodies and other targeted agents may be used alone or in combination.

4. Immunotherapy: Boosting the body’s immune response to fight cancer. CAR T-cell therapy and monoclonal antibodies are examples of immunotherapies used in NHL.

5. Radiation Therapy: High-energy beams target and destroy cancer cells. It is used when cancer is slow growing or limited to one or two spots. 

6. Stem Cell Transplantation: The procedure involves high-dose chemotherapy and an infusion of healthy stem cells. It is done to replace damaged bone marrow.

   Providers usually perform autologous (patient’s own cells) transplants. Rarely, they may perform allogeneic (from a donor) transplants.

Non-Hodgkin’s lymphoma has both common and rare types. The non-Hodgkin’s lymphoma types have different characteristics with varying treatment responses.

Recognising these types can help your medical team better tackle the disease through personalised treatments. If you have chronic symptoms or have worries about your health, you should see a healthcare expert for proper evaluation and diagnosis.

HexaHealth offers comprehensive support to NHL patients through symptom management and access to leading specialists. Using advanced technologies, the team at HexaHealth helps diagnose the type of NHL, empowering individuals to make informed decisions about their care. To know more, schedule an appointment TODAY! 

Suggested read