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Non-Hodgkin's Lymphoma

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Cellular Classification of Adult Non-Hodgkin Lymphoma

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PTLD

Patients who undergo transplantation of the heart, lung, liver, kidney, or pancreas usually require life-long immunosuppression. This may result in PTLD in 1% to 3% of recipients, which appears as an aggressive lymphoma.[236] Pathologists can distinguish a polyclonal B-cell hyperplasia from a monoclonal B-cell lymphoma; both are almost always associated with EBV.[237] Poor performance status, grafted organ involvement, high IPI, elevated LDH, and multiple sites of disease are poor prognostic factors for PTLD.[238,239] In some cases, withdrawal of immunosuppression results in eradication of the lymphoma.[240,241] When this is unsuccessful or not feasible, a trial of rituximab may be considered, because it has shown durable remissions in approximately 60% of patients and a favorable toxicity profile.[241,242] Sometimes, a combination of acyclovir and interferon-alpha has been used.[236,243] If these measures fail, doxorubicin-based combination chemotherapy is recommended, though most patients can avoid cytotoxic therapy.[244] Localized presentations can be controlled with surgery or radiation therapy alone. These localized mass lesions, which may grow over a period of months, are often phenotypically polyclonal and tend to occur within weeks or a few months after transplantation.[237] Multifocal, rapidly progressive disease occurs late after transplantation (>1 year) and is usually phenotypically monoclonal and associated with EBV.[245] These patients may have durable remissions using standard chemotherapy regimens for aggressive lymphoma.[245,246,247] Instances of EBV-negative PTLD occur even later (median, 5 years posttransplant) and have particularly poor prognoses.[248] A sustained clinical response after failure from chemotherapy was attained using an immunotoxin (anti-CD22 B-cell surface antigen antibody linked with ricin, a plant toxin).[249] An anti-interleukin-6 monoclonal antibody is also under clinical evaluation.[250]

True histiocytic lymphoma

True histiocytic lymphomas are very rare tumors that show histiocytic differentiation and express histiocytic markers in the absence of B-cell or T-cell lineage-specific immunologic markers.[251,252] Care must be taken with immunophenotypic tests to exclude ALCL or hemophagocytic syndromes caused by viral infections, especially EBV. Therapy is modeled after the treatment of comparably staged diffuse large cell lymphomas, but the optimal approach remains to be defined.

Primary effusion lymphoma

Primary effusion lymphoma presents exclusively or mainly in the pleural, pericardial, or abdominal cavities in the absence of an identifiable tumor mass.[253] Patients are usually HIV-seropositive, and the tumor usually contains Kaposi sarcoma-associated herpes virus/human herpes virus 8. Therapy is usually modeled after the treatment of comparably staged diffuse large cell lymphomas, but the prognosis is extremely poor.

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