MANTLE CELL LYMPHOMA (MCL)
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ALTERNATE NAMES
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MCL; LCM; Mantle zone lymphoma |
DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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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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Swollen lymph nodes in the neck, armpits, or groin;
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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
ICD-10: C83.10
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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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.
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Suggested
Listings for
Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.05 D
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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.
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113.05 D
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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.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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