PBL; Plasmatic Lymphoma

Plasmablastic lymphoma (PBL) is an uncommon but aggressive subtype of diffuse large B-cell lymphoma.

PBL typically presents as a mass in one or more extra‐nodal sites, usually the oral cavity or gastro‐intestinal tract. The primary organs involved are usually the gastrointestinal system, lymph nodes, oral mucosa and sometimes the skin. Nodal disease without extra‐nodal involvement is rare. These sites include the genitourinary tract, central nervous system, bone, liver, nasal cavities, lung, and orbits.

PBL can occur in people of all ages, and typically occurs in immunosuppressed adults, particularly those with HIV infection or receiving immunosuppressive therapy. PBL is rare in children. The exact underlying cause of PBL is not known.

Diagnostic testing: The diagnosis of PBL is difficult because its features overlap with myeloma and lymphoma. The diagnosis of PBL is made by:

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  Physical examination and history;

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  Blood chemistry studies;

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  Bone marrow aspiration and biopsy;

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  Computerized tomography (CAT) scan;

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  Complete blood count (CBC);

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  Cytogenetic analysis;

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  Flow cytometry;

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  Immunohistochemistry;

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  Immunophenotyping;

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  Lymph node biopsy;

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  Magnetic resonance imaging (MRI); and

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  Positron emission tomography (PET) scan.

Physical findings: The physical findings of PBL include:

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  Disseminated bone involvement;

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  Drenching night sweats;

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  Fatigue;

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  Fever (for no known reason);

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  Pain in the chest, abdomen, or bones (for no known reason);

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  Painless swelling in the lymph nodes;

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  Skin rash or itchy skin; and

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  Weight loss (for no known reason).

ICD-9: 200.8

ICD-10: C83.3

PBL is a rare disease, with male predominance. It is a most common lymphoma in HIV patients and immunosuppressed adults. This disease mostly occurs in adults and is rare in children. The median age at diagnosis is 50 years of age.

PBL is fast-growing and may become resistant to treatment. It has a very aggressive disease course comprising relapses and refractoriness to chemotherapy.

The prognosis is usually poor for individuals with PBL. Medial survival is 7 to 12 months with 2-year survival about 40 percent, and 3-year survival 10 to 25 percent.

PBL is a very aggressive lymphoma that is difficult to diagnose and treat. It requires an interprofessional team with a medical oncologist, infectious disease physician, pharmacist, and oncology nurse for treatment in the form of high dose lymphoma chemotherapy. Palliative care is considered early during treatment to help individuals with PBL to transition their care, if needed, to the hospice setting due to a very poor prognosis associated with this lymphoma.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment; and

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

Suggested Listings for Evaluation:

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