Antiviral therapy with IV acyclovir or ganciclovir has been used to inhibit EBV
replication in an effort to treat PTLD. Response is variable and may depend on the
type and extent of PTLD. A.L. already has received ganciclovir for 4 weeks during
which time she presented with PTLD. Surgery, radiation therapy, and chemotherapy
are used to treat PTLD depending on the situation. Interferon and immunoglobulin
have been effective in a few cases that appeared unresponsive to other therapies.
Monoclonal or immunoblastic, disseminated, rapidly progressive PTLD responds
poorly to traditional therapy and has a mortality rate as high as 70%. Rituximab (an
anti-B-cell, anti-CD20 antibody) is considered first-line therapy for CD20-postive
B-cell PTLD along with reduction or withdrawal of immunosuppression if possible.
Patients usually get 375 mg/m2 weekly for 4 weeks; some groups have used
prolonged therapy. Patients may have relapse or disease progression that may
respond to chemotherapy regimens such as cyclophosphamide, adriamycin,
bleomycin, vincristine, and methotrexate (PROMACE-cytaBOM).
Transplant recipients have a higher risk of myelotoxic side effects, depending on
their maintenance immunosuppression. A.L.’s prognosis is poor given the type and
extent of her PTLD, which would have been more responsive to therapy if it had been
diagnosed early. Polyclonal PTLD responds well to reduction or discontinuation of
immunosuppression and high-dose acyclovir or ganciclovir therapy for several
weeks to months. The roles of prophylactic antivirals, immunoglobulins, and EBV
PCR monitoring in the prevention of PTLD are currently controversial, with mixed
results in current literature.
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