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Primary Central Nervous System Lymphoma: A Rare Post Lung Transplantation Lymphoproliferative Disorder FREE TO VIEW

Jordanna Hostler*, MD; Anne Brown, MD; Shahzad Ahmad, MD; Steven Nathan, MD; Oksana Shlobin, MD
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Walter Reed National Military Medical Center, Bethesda, MD

Chest. 2012;142(4_MeetingAbstracts):1040A. doi:10.1378/chest.1390677
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SESSION TYPE: Miscellaneous Cases III

PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION: Lung transplantation is increasingly common with growing numbers of procedures performed and longer life expectancies for transplant recipients. There are a variety of infectious and non-infectious complications seen in the post-transplant period. One such complication is post-transplant lymphoproliferative disease (PTLD), which occurs in 2-9% of lung transplant recipients. PTLD can manifest along a spectrum ranging from cellular hyperplasia to malignant lymphoma. More than 90% of these lesions are associated with Epstein-Barr virus. It is hypothesized that EBV drives tumor formation in B-cells in the setting of post-transplant immunosuppression.

CASE PRESENTATION: This is a 63-year-old woman who underwent left single lung transplantation for non-specific interstitial pneumonia. She had a post-operative course complicated by primary graft dysfunction, pulmonary embolism, acute rejection, and recurrent infection of her left pleural space. Her post-transplant immunosuppression included cyclosporine, prednisone and mycophenylate. Nine months after transplant she developed temporal headaches, nausea, vomiting, and gait instability. Head CT revealed a large left-sided cerebellar mass; MRI confirmed a 3 cm ring-enhancing mass. Immune cell function assay at that time was 175 ng/mL ATP (less than 225 ng/mL is low). She underwent left suboccipital craniectomy for tumor resection. Biopsy showed primary central nervous system (CNS) diffuse large B-cell lymphoma with +EBV staining consistent with monoclonal PTLD. Her peripheral EBV viral load was undetectable, but she was EBV/CMV+ at transplant with an EBV/CMV + donor. The patient’s functional status was too poor to tolerate chemotherapy. Shortly after referral to palliative care she expired.

DISCUSSION: PTLD presenting as primary CNS lymphoma is rarely described in lung transplant patients; our literature search reveals four reported cases. Symptoms are vague with headache and mental status changes being most common. Imaging most commonly reveals a ring-enhancing or homogenous mass. General treatment of PTLD involves immune reconstitution, anti-viral therapy, radiotherapy, and chemotherapy. Rituximab is the chemotherapeutic agent of choice for PTLD; however, it does not penetrate the blood brain barrier. R-CHOP, methotrexate, temozolomide have been used to treat primary CNS PTLD.

CONCLUSIONS: As lung transplantation becomes increasingly prevalent the community pulmonologist should become more aware of complications including PTLD. Early consultation with a multi-disciplinary team is needed to be able to treat this devastating complication.

1) Cavaliere R et. al. "Primary Central Nervous System Post-Transplantation Lymphoproliferative Disorder" Cancer 2010;116:863-70.

2) Phan TG et. al. "Post-Transplant Primary CNS Lymphoma" Neuro Oncol, 200:2:229-238

DISCLOSURE: The following authors have nothing to disclose: Jordanna Hostler, Anne Brown, Shahzad Ahmad, Steven Nathan, Oksana Shlobin

No Product/Research Disclosure Information

Walter Reed National Military Medical Center, Bethesda, MD




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