What is Nodal Marginal Zone B cell Lymphoma

Lymphoma is divided into 2 broad groups: Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. Non-Hodgkin’s lymphoma has many subtypes – and one of them is Nodal Marginal Zone B-cell Lymphoma. As the name suggests, it primarily affects the B cells. Nodal marginal zone B-cell lymphoma is the nodal (i.e. happens in the lymph nodes) manifestation of MALT lymphoma (see other article on MALT lymphoma).
Lymphoma means cancer of the lymphatic system. The lymphatic system is important to our immune system where it helps to combat infection. The lymphatic system consists of several organs, e.g. lymph nodes, spleen, thymus and bone marrow, where all these are connected by tiny vessels. Lymphatic fluid is the medium that circulates in these tiny vessels. Like blood, it has cells in them. These cells can be divided into 2 types: T cells and B cells.

Statistics on Nodal Marginal Zone B cell Lymphoma

The following figures are according to the Australian Institute of Health and Welfare:

  • The incidence of non-Hodgkin’s lymphoma is increasing over the years.
  • In 2001, the 3499 cases of non-Hodgkin’s lymphoma were reported.
  • For non-Hodgkin’s lymphoma, the male:female ratio is 1.22:1
  • In 2000, 2938 cases of B cell lymphoma (i.e. a subgroup of non-Hodgkin’s lymphoma affecting primarily B lymphocytes) were reported in Australia. B cell lymphoma is the commonest subgroup of non-Hodgkin’s lymphoma.Nodal marginal zone B-cell lymphoma is a rare disorder. It represents 1% of all non-Hodgkin’s lymphoma.

    Risk Factors for Nodal Marginal Zone B cell Lymphoma

    The cause of nodal marginal zone B-cell lymphoma is not known. However, several risk factors in terms of age, sex and race are identified based on the statistics available.
    Age: More commonly in middle aged to elderly people.
    Sex: Females are affected slightly more than males, but overall male:female ratio of non-Hodgkin’s lymphoma is 1.22:1 (see above)
    Race: Caucasians are more commonly affected than other races such as Chinese, Japanese and African.
    Some diseases like dry eye or dry mouth is associated with nodal marginal zone B-cell lymphoma.

    Progression of Nodal Marginal Zone B cell Lymphoma

    Clinically and historically, non-Hodgkin’s lymphoma can be subdivided into 2 groups: indolent (low grade) and aggressive (high grade) lymphomas. Nodal marginal zone B-cell lymphoma is under the indolent group – which means it has better prognosis but may not respond to treatment when compared to aggressive groups of lymphomas.
    As for the course of the disease, it is dependent on the clinical stage when the disease is diagnosed.
    The staging for non-Hodgkin’s lymphoma (which is applicable for nodal marginal zone B-cell lymphoma as well) can be summarised as below:
    Stage I – involvement of a single lymph node region or organ or site other than lymph nodes
    Stage II – 2 or more lymph node regions involved on the same side of diaphragm
    Stage III – 2 or more lymph node regions involved on both sides of diaphragm
    Stage IV – one or more organs (eg bone marrow) involved
    Each stage is subclassified into A and B (eg IIA), where B means presence of B symptoms (see below) while A means abscence of the following symptoms:

  • Unexplained fever > 38 Centigrade
  • Unexplained weight loss (defined as >10% of body weight in 6 months)
  • Night sweats

    How is Nodal Marginal Zone B cell Lymphoma Diagnosed?

    The usual basic blood tests will be done, including those to look at the blood and other blood components. Other general tests will determine the function of the liver.

    Prognosis of Nodal Marginal Zone B cell Lymphoma

    Generally speaking, nodal marginal zone B-cell lymphoma has a very good prognosis.
    The prognosis is dependent on the clinical stage when the disease is diagnosed. The later the stage, the worse the prognosis.
    Other than staging of the disease, the following criteria are used to determine the prognosis of non-Hodgkin’s lymphoma (that includes nodal marginal zone B-cell lymphoma).

    • Age >60
    • Serum lactate dehydrogenase (LDH) concentration greater than normal – this is a biochemical compound found in the blood
    • Patient is having symptoms and in bed <50% of the day or worse than this
    • Stage III or IV (see above for explanation on staging)
    • More than 1 involved site other than lymph nodesThe more the criteria are met, the worse the prognosis.

      How is Nodal Marginal Zone B cell Lymphoma Treated?

      Some of the disease grow slowly and cause no symptoms to the patients for many years. Watchful monitoring may be the choice of therapy, but if the disease starts to produce symptoms then treatment can be given promptly.
      The following treatment options are usually applied, either alone or in combination.

    • Chemotherapy: this is using chemical (drugs) to kill the cancerous cells.
    • Radiotherapy: this is using radiation to kill the cancerous cells. This is used when there is one or more lymph node regions affected in the same part of the body.
    • Steroids: these are the drugs often used with chemotherapy in order to reduce its side effects, for example, nausea and vomiting, etc.
    • Stem cell transplantation: this is first killing the cancer and normal lymphatic cells with chemotherapy and radiation therapy, then infusing the body with non-cancerous new stem cells (cells that are precursor to the lymphocytes in the body). However, this is limited to selected patients only due to the side effect profile of such treatment.

      Nodal Marginal Zone B cell Lymphoma References

      [1] Cancer in Australia 2001 [online]. 2004. [Cited 2005 September 13th]. Available from: URL: http://www.aihw.gov.au/publications/can/ca01/ca01-c03.pdf
      [2] Clinical and pathologic features of the marginal zone lymphomas [online]. 2005. [Cited 2005 September 13th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=lymphoma/15507&type=A&selectedTitle=1~2
      [3] How common is lymphoma [online]. 2003. [Cited 2005 September 13th]. Available from: URL: http://www.lymphoma.org.au/content/?id=25


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