MANTLE CELL LYMPHOMA (MCL)

Mantle Cell Lymphoma (MCL) is an aggressive (fast-growing) type of B-cell non-Hodgkin's lymphoma. Non-Hodgkin's lymphomas are related malignancies (cancers) that affect the lymphatic system. It is marked by small to medium-size cancer cells that may be in the lymph nodes, spleen, bone marrow, blood, and gastrointestinal system. MCL is a B-cell lymphoma that develops from malignant B-lymphocytes within a region of the lymph node known as the "mantle zone." MCL results from errors in the production of a lymphocyte or transformation of a lymphocyte into a malignant cell. Abnormal, uncontrolled growth and multiplication (proliferation) of malignant lymphocytes may lead to enlargement of a specific lymph node region or regions; involvement of other lymphatic tissues, such as the spleen and bone marrow; and spread to other bodily tissues and organs, potentially resulting in life-threatening complications. The specific symptoms and physical findings may vary from case to case, depending upon the extent and region(s) of involvement and other factors. MCL primarily affects men over the age of 50 years. Many affected individuals have widespread disease at diagnosis, with involved regions often including multiple lymph nodes, the spleen, and, potentially, the bone marrow, the liver, and/or regions of the digestive (gastrointestinal) tract.

Diagnostic testing: MCL diagnosis is obtained by pathologic review of a lymph node biopsy or bone marrow specimen. This usually includes flow cytometry testing and chromosomal analysis, which show CD5-positive cells, cyclin D1 protein over expression, and translocation of chromosomes 11 and 14.

Physical findings: Some physical findings with MCL may include:

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  Loss of appetite and weight;

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  Fever;

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  Night sweats;

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  Nausea or vomiting;

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  Swollen lymph nodes in the neck, armpits, or groin;

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  Heartburn;

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  Belly pain;

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  Bloating; and

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  A sense of fullness or discomfort from enlarged tonsils, liver or spleen.

ICD-9: 200.3, 200.4, 200.40, 200.41, 200.42, 200.43, 200.44, 200.45, 200.46, 200.47, 200.48, 202.8

MCL is characterized by an extremely poor prognosis. This cancer has the shortest average survival of all lymphoma types. Long-time survivors are rare, only very few patients with an overall survival over 10 years have been reported. Since MCL is expected to recur no matter what chemotherapy is used, viable lymphoma (microscopic in many cases) must be presumed to persist after chemotherapy even when the patient has an initial complete remission.

Chemotherapy and radiation therapy are used for palliation with variable degrees of success. Stem cell transplantation (SCT) and bone marrow transplantation (BMT) are also treatment options. There is no cure for MCL. However, these treatments could lead to an increase in life expectancy for some individuals.

Suggested MER for Evaluation:

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  The diagnosis of MCL is usually based on the pathology report from a lymph node or bone marrow specimen.

Suggested Listings for Evaluation:

REMARKS

A pathology report is the only evidence required to adjudicate the listing.

In cases where there is a SCT/BMT, listing 13.05 D should be used instead of 13.05 C. MCL has a poor prognosis and high chance of relapse even in the event of a transplant.

A pathology report is the only evidence required to adjudicate the listing.

In cases where there is a SCT/BMT, listing 113.05 D should be used instead of 113.05 C. MCL has a poor prognosis and high chance of relapse even in the event of a transplant.