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Mature B-Cell Lymphomas

Page Sections 

Follicular Lymphoma

Mantle Cell Lymphoma

Diffuse Large B-Cell Lymphoma (DLBCL)

Burkitt's Lymphoma

Chronic Lymphocytic Leukaemia (CLL)/Small Lymphocytic Lymphoma (SLL)

Marginal Zone Lymphomas

Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphatic Tissue (MALT) Type

Splenic Marginal Zone Lymphoma (SMZL)

Nodal Marginal Zone Lymphoma (NMZL)

Lymphoplasmacytic Lymphoma


 

Follicular Lymphoma

What is it?

  • Follicular lymphoma is a B-cell lymphoma arising from B-lymphocytes. The tumour cells often create a circular or follicular pattern when viewed under the microscope.
  • Follicular lymphoma is the most common sub-type of indolent (slow-growing) NHL, comprising 20% to 30% of all NHL.
  • Follicular lymphoma typically affects middle-aged or older adults.
  • Like most indolent lymphomas, people diagnosed with follicular lymphoma usually have tumours in many parts of the body at the time of diagnosis.
  • Follicular lymphomas can transform into a more aggressive form of NHL, usually a diffuse large B-cell lymphoma.

What are the symptoms?

  • The most common sign of follicular NHL is painless swelling in the lymph nodes of the neck, armpit or groin. Sometimes more than one group of nodes is affected.
  • The diagnosis of follicular NHL is confirmed by a lymph node biopsy. Other tests including X-rays, bone marrow biopsy, CT scans and blood tests may also be performed.
  • In the event that follicular NHL transforms to a more aggressive form of NHL, the NHL must then be re-diagnosed and a second lymph node biopsy or other tests may be required.

How is it treated?

Treatment for follicular lymphoma depends on the stage of the lymphoma.

  • Early stage: People who are diagnosed at an early stage (stage 1 or 2) may receive no treatment (Watchful Waiting approach), radiation therapy or chemotherapy. Local radiation therapy has resulted in remissions lasting longer than 10 years in 50% of people.
  • Later stage: People who are at a later disease stage (stage 3 or 4) at the time of diagnosis but who are not experiencing symptoms may receive no treatment (Watchful Waiting approach) with very close monitoring. Once the need for treatment arises the most common treatments include:
    • Single-agent chemotherapy (e.g., chlorambucil) or combination chemotherapy (e.g. CVP or CHOP)
    • MabThera® (rituximab), a monoclonal antibody, is often used either alone or in combination with chemotherapy
    • Radiation therapy
    • Prolonged treatment with MabThera (called MabThera maintenance therapy) may also be used to treat people with follicular NHL who have received treatment for follicular lymphoma and have achieved remission (complete or partial remission). This prolonged administration with MabThera maintenance therapy (generally administered every three months for a period of two years) has been shown to sustain the response obtained from the initial therapy and may improve survival for people with follicular lymphoma.

Follicular NHL usually responds quite well to chemotherapy. However, there is a risk that it may return in future years. At that time, treatment is given again with the aim of achieving remission again. This pattern may repeat itself over many years.or some patients with relapsed FL, high-dose chemotherapy and an autologous stem cell transplant (in which patients receive their own stem cells) or an allogeneic stem cell transplant (in which patients receive stem cells from a donor) may provide a prolonged disease-free interval.

Treatments Under Investigation

Many treatments are currently being tested in clinical trials for patients who are newly diagnosed or have relapsed/refractory FL. For example, rituxumab plus lenalidamide (Revlimid) is being assessed in treatment-naive patients, and Y90 ibritumomab tiuxetan is being investigated as consolidation therapy after R-CHOP in high-risk patients. Combinations of treatment modalities, immunochemotherapy, ibritumomab tiuxetan, and bone marrow transplant, are being investigated for helping patients have prolonged disease-free survival/remission.

Other treatments under investigation include:

  • Bortezomib (Velcade)
  • Everolimus (Afinitor)
  • Idelalisib (GS-1101, formerly CAL-101)
  • Ibrutinib (PCI-32765)
  • Lenalidomide (Revlimid)
  • Ofatumumab (Arzerra)
  • Panobinostat 

It is critical to remember that today's scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with their clinician for any treatment updates that may have recently emerged.

Follow Up

Because FL is generally characterized by multiple disease relapses after responses to a variety of treatments, patients in remission should have regular visits with a clinician who is familiar with their medical history as well as with the treatments they have received. Medical tests (such as blood tests CAT and PET scans) may be required at various times during remission to evaluate the need for additional treatments.

Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care.

Lymphoma survivors should receive regular medical exams from a physician who is familiar with their medical history as well as the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. Please order your free lymphoma diary from our resource section.  

Mantle Cell Lymphoma

What is it?

  • Mantle cell lymphoma is a type of aggressive B-cell lymphoma that most commonly affects men over the age of 50 years, although it can affect women.
  • It is relatively uncommon and accounts for approximately 5% to 10% of all NHL cases.

What are the symptoms?

  • The most common symptom is a painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes. Often lymph nodes in more than one area of the body are affected. Splenomegaly (enlargement of the spleen) is relatively frequent and may cause a feeling of fullness in the abdomen after eating only small amounts.
  • Mantle cell lymphoma grows aggressively (fast) and may spread to other organs in the body, including the bone marrow, spleen and liver. It can also spread to the stomach or digestive tract.
  • Mantle cell lymphoma is usually widespread at the time of diagnosis. Lymph node biopsy is used to confirm the diagnosis. Other tests including X-rays, bone marrow biopsy, CT scans and blood tests may also be performed.

How is it treated?

  • Mantle cell lymphoma is usually treated with combination chemotherapy (such as CHOP chemotherapy) or combination chemotherapy (e.g. CHOP plus MabThera). Mantle cell lymphoma can also be treated with radiation therapy, stem-cell transplant and other newer treatments.
  • Treatment is often initially successful. However, mantle cell lymphoma frequently relapses. While treatments have been developed to treat this type of lymphoma, people with mantle cell lymphoma are often encouraged to participate in clinical trials so they can receive newer treatments that are not yet available in clinical practice.

Diffuse Large B-Cell Lymphoma (DLBCL)

What is it?

  • DLBCL is an aggressive B-cell NHL and is the most common type of NHL accounting for 30% to 40% of all cases.
  • The average age of diagnosis for DLBCL is the mid-sixties, however this cancer can also affect children.
  • Mediastinal large B-cell lymphoma is a sub-type of DLBCL, where the cancer arises in the thymus gland and lymph nodes behind the mediastinum (the area in the middle of the chest, between the lungs). Mediastinal large B-cell lymphoma can lead to symptoms of shortness of breath, cough and pain in the chest and cause swelling of the neck, arms and face due to the swollen lymph nodes pressing on the veins in the chest (this swelling is known as “superior vena cava obstruction”). This form of DLBCL may occur at any time from early adulthood to old age but is most common between the ages of 25 and 40 years. It is twice as common in women as in men.

What are the symptoms?

  • The most common symptom of DLBCL is a painless swelling in the neck, armpit or groin caused by enlarged lymph nodes. Often lymph nodes in more than one area of the body are affected.
  • Usually, the disease is widespread at the time of diagnosis, with symptoms including weight loss, fever and night sweats.
  • About 50% of people have organ involvement at the time of diagnosis with the most common organs involved being the digestive (gastrointestinal) tract and the bone marrow.
  • The diagnosis of DLBCL is confirmed by a lymph node biopsy. When looked at under a microscope, the tumour cells of DLBCL appear large in size and display a diffuse or scattered pattern. Other tests including X-rays, bone marrow biopsy, CT scans and blood tests may also be performed.

How is it treated?

  • The standard treatment for DLBCL is MabThera plus CHOP chemotherapy. Other therapies include radiation therapy, stem-cell transplants and steroid therapy. This type of aggressive NHL is very sensitive to treatment and a large percentage of people with DLBCL can be cured.

Burkitt’s Lymphoma

What is it?

  • Burkitt’s lymphoma is a very aggressive form of NHL and commonly affects both children and adults, with males being affected more frequently than females.
  • The disease may be associated with viral infection such as the human immunodeficiency virus (HIV) and the Epstein-Barr virus.
  • Burkitt’s lymphoma accounts for 30% to 40% of all childhood lymphomas and occurs in children between the ages of 5 and 10 years and in adults between the ages of 30 and 50 years.

What are the symptoms?

  • The most common symptoms are swollen lymph nodes and abdominal swelling.
  • Burkitt’s lymphoma may also affect other organs such as the eyes, ovaries, kidneys, central nervous system and glandular tissue such as breast, thyroid or tonsil. Disease in these organs may cause variable symptoms.
  • The diagnosis of Burkitt’s lymphoma is confirmed by a lymph node biopsy. Other tests including X-rays, bone marrow biopsy, CT scans and blood tests may also be performed.

How is it treated?

  • Although Burkitt’s lymphoma has a very aggressive course, survival rates with treatment are approximately 80%. The most common treatment for Burkitt’s lymphoma is intensive chemotherapy. Other treatments include monoclonal antibody therapy and stem-cell transplants.

Chronic Lymphocytic Leukaemia (CLL)/Small Lymphocytic Lymphoma (SLL)

What is it?

  • Chronic lymphocytic leukaemia (CLL) and small lymphocytic lymphoma (SLL) are B-cell lymphomas. CLL and SLL are essentially the same disease, the only difference being where the cancer primarily occurs. When most of the cancer cells are located in the bloodstream and the bone marrow, the disease is referred to as CLL, although the lymph nodes and spleen are often involved. When the cancer cells are located mostly in the lymph nodes, the disease is called SLL.

    CLL/SLL tends to be an indolent (slow-growing) cancer; however, over time, it can progress to a more aggressive type of lymphoma. Common signs of disease include a swelling of the liver and spleen and enlargement of the lymph nodes in the neck, underarm, stomach, or groin. Other symptoms of CLL/SLL can include fatigue, shortness of breath, anaemia, bruising, night sweats, weight loss, and frequent infections. Often, people with CLL/SLL have no obvious symptoms of the disease at diagnosis. Approximately one-third of all CLL/SLL patients will live for years without symptoms.

What are the symptoms?

  • Symptoms include swollen lymph nodes and an enlarged spleen (splenomegaly), which can cause a mass under the left ribcage, fullness in the abdomen and weight loss. Fatigue, recurrent minor infections or bleeding episodes may also be experienced.
  • SLL is usually diagnosed by lymph node biopsy. A bone marrow biopsy may be performed if there is minor lymph node involvement and reduced blood cell numbers.

How is it treated?

  • Initial treatment is often Watch and Wait. Some patients may never require therapy, whilst patients who develop symptoms of advancing disease receive therapy in almost all cases. MabThera is now commonly used in combination with chemotherapy for the treatment of CLL/SLL.
  • Other treatments include chemotherapy with drugs such as chlorambucil and fludarabine or a combination chemotherapy regimen used in other lymphomas such as CVP or CHOP chemotherapy. Younger patients with this disease may be candidates for bone marrow transplant.

Treatment is based on the severity of associated symptoms as well as the rate of cancer growth. If patients show no or very few symptoms, doctors may decide not to treat it right away, an approach referred to as "watch and wait" or "watchful waiting." Studies have shown that suitable patients who follow a "watch and wait" approach have outcomes similar to those being treated early in the course of their disease.

Some factors that influence the decision to treat patients with CLL include:

  • Relatively rapid increase of blood lymphocyte counts
  • Enlarging lymph nodes
  • Enlarging spleen
  • Worsening anemia
  • Falling platelet count

*Several of these factors are often present at the same time.

There are many current frontline treatment options for CLL/SLL. In general, patients who are older or frail are typically treated with less aggressive regimens than those who are younger and healthier. Stem cell transplantation is a treatment option for certain patients, including those with advanced disease, those with certain high-risk features, or those whose disease has transformed into a more aggressive form. Typically stem cells from a donor are used (called an allogeneic stem cell transplant) but with less toxic treatments as preparation (sometimes called a mini-allogeneic transplant)

The Institute for choice at the University of South Australia is conducting important research to assist with making new CLL treatments available in Australia, click here to find out how you can help.   

Common combination drug regimens used to treat CLL/SLL include: ( Not all of the below are available in Australia)

 

Drug or Regimen Abbreviation

Generic Name (Brand Name)

B

Bendamustine

BR

Bendamustine (Treanda)

Rituximab (Rituxan)

2CDA

Cladribine (Leustatin)

C

Cyclophosphamide (Clafen, Cytoxan, Neosar)

Ch 

Chlorambucil (Leukeran) 

 CvPred-R

Cyclophosphamide (Clafen, Cytoxan, Neosar)

Prednisone (Deltasone)

Rituximab (Rituxan) 

Fludarabine (Fludara) 

FR 

Fludarabine (Fludara)

Rituximab (Rituxan)

 FCR

 Fludarabine (Fludara)

 Cyclophosphamide (Clafen, Cytoxan, Neosar)

Rituximab (Rituxan) 

 HDMPR

High-dose methylprednisolone (Depo-Medrol, Medlone 21, Medrol, Meprolone, Metrocort, Metypred, Solu-Medrol, Summicort)

Rituximab (Rituxan

 

 Ibrutinib

(alone)

Ibrutinib (Imbruvica) 

Idelalisib

plus 

Idelalisib (Zydelig) 

Rituximab 

Rituximab (Rituxan) 

Obinutuzumab

plus Chlorambucil

Obinutuzumab (Gazyva)

Chlorambucil (Leukeran)

 OFAR

Oxaliplatin (Eloxatin)

Fludarabine (Fludara)

Cytarabine (Cytosar-U, Tarabine PFS)

Rituximab (Rituxan) 

Ofatumumab

(alone)

Oatumumab 

Ofatumumab plus

Chlorambucil

Ofatumumab (Arzerra)

Chlorambucil (Leukeran)

PCR 

Pentostatin (Nipent)

Cyclophosphamide (Clafen, Cytoxan, Neosar)

Rituximab (Rituxan) 

RCHI 

Rituximab (Rituxan)

Chlorambucil (Leukeran)

RCHOP 

Rituximab (Rituxan)

Cyclophosphamide (Clafen, Cytoxan, Neosar)

Doxorubicin/hydroxydaunorubicin (Ardiamycin, Rubex)

Vincristine (Oncovin, Vincasar PFS)

Prednisone (Deltasone) 

Marginal Zone Lymphomas

  • Marginal zone lymphomas are a type of indolent B-cell lymphoma that account for approximately 10% of all NHL cases. Marginalzone lymphomas can be categorised according to the area affected:
    • Mucosa-associated lymphatic tissue (called MALT lymphoma), which can affect the gastrointestinal tract, eyes, thyroid, salivary glands, bladder, kidney, lungs, neurological system or skin.
    • Spleen (called splenic marginal zone B-cell lymphoma)
    • Lymph nodes (called nodal marginal zone B-cell lymphoma).
    • The average age of diagnosis of marginal zone lymphoma is 65 years, although MALT lymphomas can occur earlier.

Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphatic Tissue (MALT) Type

What is it?

  • MALT lymphomas mainly occur outside the lymph nodes, in extranodal sites. People who develop this type of NHL often have a separate autoimmune disease or inflammatory process such as Sjögren’s syndrome (salivary gland MALT), Hashimoto’s thyroiditis (thyroid MALT), or Helicobactergastritis (gastric MALT).

What are the symptoms?

  • Symptoms may include upper abdominal discomfort or local symptoms relating to where the disease occurs. The diagnosis is made by biopsy of the tumour for confirmation of small B-cell infiltration in the tumour.

How is it treated?

  • MALT type lymphoma is often curable when the tumour is localised. Surgery is not often a common treatment for NHL however in MALT lymphoma therapies such as surgery or radiation therapy can be curative. People with more extensive disease are usually treated with single-agent chemotherapy such as chlorambucil or combination chemotherapy. People with gastric MALT lymphomas who are infected with bacteria called Helicobacter pylori can achieve long remission in the majority of cases once the infection is effectively treated with antibiotics. 
  • In some cases this type of NHL can transform into the more aggressive diffuse large B-cell lymphoma (DLBCL). The standard therapy for DLBCL is CHOP chemotherapy plus MabThera.

Splenic Marginal Zone Lymphoma (SMZL)

What is it?

  • SMZL is a type of indolent B-cell NHL that predominantly involves the spleen. 
  • It is a rare type of lymphoma accounting for less than 1% of all NHL. It most commonly occurs in adults and slightly more frequent in women than in men.

What are the symptoms?

  • Symptoms do not normally appear until years after the disease has begun. The most common symptom is an enlarged spleen (splenomegaly). Unlike many other types of NHL, there are normally no swollen lymph nodes.
  • There is usually involvement of the blood and bone marrow at the time of diagnosis, with the only other symptom being fatigue.
  • Diagnosis is based on identification of the cell type together with the typical clinical findings. A biopsy of the bone marrow can often confirm diagnosis however removal of the spleen (splenectomy) is occasionally required for tissue examination.

How is it treated?

  • A number of different approaches may be taken with SMZL, including Watchful Waiting, removal of the spleen (splenectomy), radiation therapy, chemotherapy and biologic therapies with drugs such as MabThera.

Nodal Marginal Zone Lymphoma (NMZL)

What is it?

  • NMZL is a type of indolent B-cell NHL that is mainly confined to the lymph nodes. 
  • It is a rare form of lymphoma, accounting for only 1% to 3% of all NHL cases.

What are the symptoms?

  • The most common symptom is a painless swelling in the neck, armpit or groin caused by enlarged lymph nodes. Sometimes more than one group of nodes is affected.
  • NMZL is diagnosed by lymph node biopsy.

How is it treated?

  • The most common treatments for NMZL include Watchful Waiting, radiation therapy, chemotherapy with medications such as chlorambucil or fludarabine, and MabThera (often used in combination with chemotherapy).

Lymphoplasmacytic Lymphoma

(Also Called Waldenstrom’s Macroglobulinemia or Immunocytoma)

What is it?

  • Lymphoplasmacytic lymphoma is a rare form of B-cell lymphoma, making up 1% to 2% of all NHL cases and typically affecting older adults.
  • It has a slow-growing, indolent course arising from mature B-cells that are on their way to developing into plasma cells (B-cells that produce antibodies).
  • In this type of lymphoma there is an overproduction of a certain type of antibody, called IgM. When this IgM antibody is present, the lymphoma is also referred to as Waldenstrom’s macroglobulinemia. A large amount of IgM in the bloodstream causes thickening (hyperviscosity) of the blood.

What are the symptoms?

  • Lymphoplasmacytic lymphoma normally develops over a long period of time. Symptoms are not usually very obvious and the disease is often found by chance when getting a routine blood test or an examination for some other reason.
  • Symptoms may include weakness, fatigue and bruising as a result of altered blood cell levels. Lymph nodes may be enlarged, as may the liver and spleen.
  • Because there may be a thickening of the blood when the IgM antibody is present, this can cause other symptoms including sight problems, headaches, hearing loss or confusion.
  • This type of NHL is usually suspected after an abnormal blood test. Other tests that help confirm the diagnosis include bone marrow biopsy, ultrasound and/or CT scan (to determine whether the spleen or liver are enlarged).

How is it treated?

  • Treatment of lymphoplasmacytic lymphomas may include chemotherapy (such as chlorambucil or fludarabine), combination chemotherapy (such as CVP), MabThera (with or without chemotherapy) or surgery (to remove the spleen). A procedure called plasma exchange (or plasmapheresis) may be used to treat the blood thickening (hyperviscosity) associated with this disease.