Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
June 2020
Next Case of the Month Coming July 7...
Epstein-Barr Virus–Associated Post-Transplant Lymphoproliferative Disorders
- Background:
- Post-transplant lymphoproliferative disorders (PTLDs) are recognized as potential, fatal complications following solid organ or allogeneic hematopoietic cell transplantation as a result of immunosuppression.
- Up to 90% of PTLD cases are associated with Epstein-Barr virus (EBV).
- The median time from transplant to PTLD is approximately 4.4 years.
- CNS involvement in PTLD is a poor prognostic factor.
- Clinical Presentation:
- A previously EBV-seronegative patient during immunosuppression presenting with new EBV infection poses significant risk for the development of PTLD.
- Presenting symptoms usually include focal neurologic symptoms depending on the location of lesions, seizures, and headaches.
- Key Diagnostic Features:
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Similar to other immunocompromised patients with lymphoma, patients typically present with a single or multiple ring-enhancing brain lesions.
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EBV-associated PTLD tends to be a more angiocentric and angiodestructive process and frequently shows extensive necrosis, which is rare in immunocompetent patients with primary CNS lymphoma.
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Parenchymal hemorrhage is not uncommon, whether it is spontaneous or postbiopsy.
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Histologic diagnosis of primary CNS-PTLD requires biopsy confirmation and exclusion of opportunistic infections such as toxoplasmosis.
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- Differential Diagnoses
- In our patient, symmetric deep white matter lesions as well as the bilateral temporal cortical signal abnormalities suggest watershed infarcts from hypoperfusion. Causes include sepsis, cardiac arrest, or arrhythmia. Multiple lesions with peripheral enhancement can represent subacute infarcts. These conditions, however, do not typically cause multiple areas of hemorrhage as seen in this patient.
- Various CNS infections, including HSV, can cause extensive areas of necrosis and hemorrhage.
- Opportunistic infections such as toxoplasmosis can cause multiple ring-enhancing lesions, although toxoplasmosis does not typically cause hemorrhage.
- Treatment:
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Initial treatment options are reduction of immunosuppression, systemic chemotherapy and/or monoclonal antibody therapy, whole-brain radiotherapy, immunotherapy with the anti-CD20 monoclonal antibody rituximab, or a combination of these.
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Other treatments, such as adoptive immunotherapy with EBV-specific cytotoxic T cells, are generally reserved for persistent disease despite initial therapy.
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