Program Operations Manual System (POMS)
TN 1 (10-08)
DI 23022.230 Mantle Cell Lymphoma (MCL)
COMPASSIONATE ALLOWANCE INFORMATION
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 (lymphomas). 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.|
B-cell lymphoma, Non-Hodgkin's Lymphoma
DIAGNOSTIC TESTING AND CODING
MCL diagnosis is obtained by pathologic review of a lymph node biopsy and/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.
The treatment is chemotherapy but the exact regimen of drugs may vary. Refractoriness to chemotherapy is usual and unfortunately, high-dose stem cell transplants have not shown an overall survival benefit.
Classical 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.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation: The diagnosis of MCL is usually based on the pathology report from a lymph node or bone marrow specimen.
Suggested Listings for Evaluation:
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.
Last Updated: 8/6/2015
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