PRIMARY
EFFUSION LYMPHOMA |
ALTERNATE NAMES
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PEL; Body Cavity Lymphoma; Body Cavity-Based Lymphoma; AIDS – Related Lymphoma
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DESCRIPTION
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Primary Effusion Lymphoma (PEL) is a rare form of B-cell (non-Hodgkin) lymphoma. PEL originates from body cavities
such as pleural space or pericardium (both are in the chest), and peritoneum (located
in the abdomen. Signs and symptoms of PEL result from buildup of fluid in the affected
cavity. Depending on which cavity is involved, these symptoms can include shortness
of breath, chest pain, and abdominal distention. This fluid build-up leads to lung,
heart, or gastrointestinal organ dysfunction, respectively. PEL is more prevalent
in immunodeficient people. The majority of people diagnosed with PEL have been seropositive
for human immunodeficiency virus (HIV positive) for a number of years or have AIDS.
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DIAGNOSTIC TESTING, PHYSCIAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnosis is made by cytological examination of samples of fluid removed from the
pleural, pericardial, or peritoneal spaces. PEL can also be diagnosed by surgical
excision of the involved tissues or by biopsy of the body cavity lining.
Physical findings: Signs and symptoms of PEL result from buildup of fluid in the affected cavity. Depending
on which cavity is involved, these symptoms can include:
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Abdominal distention; and
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Lung, heart, or gastrointestinal organ dysfunction
ICD-9:
200.8
ICD-10: C83.8
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PROGRESSION
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PEL is an aggressive non-Hodgkin lymphoma. The prognosis of primary effusion lymphoma
is very poor with a median survival after diagnosis of 2 – 3 months without treatment
and about six months with treatment. Chemotherapy is often of short duration. Mortality
of people with PEL is frequently associated with opportunistic infection, HIV related
complications, and progression of cancer.
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TREATMENT
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There currently is no cure for primary effusion lymphoma. Treatment depends on the
age and general health of the person and the specific type and staging of the disease
at the time of diagnosis. Treatments include HAART (Highly Active Anti-retroviral
Therapy), chemotherapy, and stem cell transplantation.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Documentation of HIV infection, cytology reports of effusion fluid, or pathology reports
of biopsied body cavity lining tissue;
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Clinical description of findings;
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Reports showing the detection of HHV-8 and EBV; and
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets |
14.11
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114.11
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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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