TN 16 (03-18)
DI 23022.645 Primary Effusion Lymphoma
COMPASSIONATE ALLOWANCE INFORMATION
PRIMARY EFFUSION LYMPHOMA
PEL; Body Cavity Lymphoma; Body Cavity-Based Lymphoma; AIDS – Related Lymphoma
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.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
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.
ONSET AND PROGRESSION
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.
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.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Documentation of HIV infection, cytology reports of effusion fluid, or pathology reports of biopsied body cavity lining tissue
Clinical description of findings; reports showing the detection of HHV-8 and EBV
CT scan reports
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.