What is Mantle 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 Mantle Cell Lymphoma. It is a B-cell lymphoma, which means it primarily affects the B lymphocytes.
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 Mantle 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, there were 3499 cases of non-Hodgkin’s lymphoma reported in Australia.
  • For non-Hodgkin’s lymphoma, the male:female ratio is 1.22:1
  • In 2000, there were 2938 reported cases of B cell lymphoma (i.e. a subgroup of non-Hodgkin’s lymphoma affecting primarily B lymphocytes) in Australia. B cell lymphoma is the commonest subgroup of non-Hodgkin’s lymphoma.The male to female ratio for mantle cell lymphoma is 3 to 1.
    Most patients are in their sixties (around 63) when the disease is diagnosed.
    Of these, about 70% of the patients are in stage IV disease (see below regarding staging)

    Risk Factors for Mantle cell lymphoma

    Like most lymphomas, no causes has been found for mantle cell lymphoma. Yet based on statistics, several factors can be identified:
    Age: The disease mostly affects late middle age to elderly people
    Sex: Male predominance
    Race: Caucasians are affected more than Asians and Africans/African-Americans

    Progression of Mantle cell lymphoma

    Clinically and historically, non-Hodgkin’s lymphoma can be subdivided into 2 groups: indolent (low grade) and aggressive (high grade) lymphomas. Mantle 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 Ann-Arbor staging for non-Hodgkin’s lymphoma (which is applicable for mantle cell lymphoma) 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 (e.g. bone marrow) involvedEach stage is subclassified into A and B (e.g. IIA), where B means presence of B symptoms (see below) while A means abscence of the following symptoms:
  • Unexplained a href=”/fever/”>fever> 38 Centigrade
  • Unexplained weight loss (defined as >10% of body weight in 6 months)
  • Night sweats

    Prognosis of Mantle cell lymphoma

    Mantle cell lymphoma is considered to be an indolent non-Hodgkin’s lymphoma, which has better prognosis as compared to T cell lymphoma. Yet because 70% of the patients have stage IV disease at diagnosis (see incidence), mantle cell lymphoma has only moderate prognosis.
    The prognosis is dependent on the clinical stage when the disease is diagnosed. The later the stage, the worse the prognosis.
    The average survival for patients diagnosed with mantle cell lymphoma is 3 to 4 years.
    General investigations:
    The usual basic 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.

    How is Mantle cell lymphoma Treated?

    Mantle cell lymphoma is a B-cell lymphoma, hence it is classified as an indolent lymphoma (see above). Although some patients may survive without treatment for many years, many patients present as aggressive lymphoma rather than an indolent one.
    While ‘watchful monitoring’ may the therapy of choice in some patients that do not have any symptoms, more aggressive treatment is usually adopted for patients with mantle cell lymphoma.
    The following treatment modalities can either be given alone or in combination:
     

  • Chemotherapy: this is using chemical (drugs) to kill the cancer cells. One or more anti-cancer drugs may be used, but usually multiple drugs are used.
  • 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: this is to reduce the side effects of anti-cancer drugs such as nausea and vomiting.
  • Allogenic stem cell transplantation: this is first killing the cancer cells and normal lymphatic cells with chemotherapy and radiation, then infusing the body with non-cancerous new stem cells (cells that are precursor to the lymphatic system cells). However, this is limited to selected patients only due to the side effect profile of such treatment.
  • Monoclonal antibody therapy: monoclonal antibodies are drugs that recognise, target and attach to particular proteins on the surface of cancer cells. This can then stimulate the body’s own immune system to destroy the cell. Sometimes if the disease is aggressive, a monoclonal antibody may be added to chemotherapy. The current monoclonal antibody used is rituximab.

    Mantle cell lymphoma References

    [1] Cancer in Australia 2001 [online]. 2004. [Cited 2005 September 14th]. Available from: URL: http://www.aihw.gov.au/publications/can/ca01/ca01-c03.pdf
    [2] Clinical and pathologic features of mantle cell lymphoma [online]. 2005. [Cited 2005 September 14th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=lymphoma/15026
    [3] How common is lymphoma [online]. 2003. [Cited 2005 September 14th]. Available from: URL: http://www.lymphoma.org.au/content/?id=25
    [4] Treatment of mantle cell lymphoma [online]. 2005. [Cited 2005 September 14th]. Available from: URL: http://www.utdol.com/application/topic.asp?file=lymphoma/20074