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A6564 - When a Donor Loses Control: A Rare Case of Post-Transplant Lymphoproliferative Disorder
Author Block: C. L. Shappley1, Z. Ghandour2; 1Pulmonary/Critical Care, Tulane University, New Orleans, LA, United States, 2Pathology, Tulane University, New Orleans, LA, United States.
Post-transplant lymphoproliferative disorder (PTLD) is a common malignancy in solid organ transplant recipients and is often recipient derived. PTLD is associated with EBV positive B-lymphocytes and the loss of T-cell mediated immune response following transplant. Alemtuzumab, a potent monoclonal anti-CD52 antibody, has been introduced into clinical practice for induction following lung transplantation for its prolonged T-cell depletion. Since it also has B-cell depletion properties, Alemtuzumab should theoretically decrease early PTLD in solid organ recipients.
A 64 y/o woman who received a bilateral lung transplant from a male donor (EBV D+/R+) 75 days prior, is admitted for fevers. At time of transplant, she received induction with high dose steroids and Alemtuzumab. Post transplant course was uneventful and she was discharged on POD 15 with a standard medication regimen. Routine clinic follow-up and labs were performed on POD 19, 26, 33, and 48 which showed therapeutic Tacrolimus levels and continued improvement in symptoms and FEV1. Surveillance bronchoscopy preformed on POD 42 was without evidence of rejection or infection. On POD 67, a routine CXR showed a right hilar opacity confirmed as hilar lymphadenopathy on CT chest. She underwent an EBUS with TBNA on POD 75 which was non-diagnostic. On admission, she was started on broad spectrum antibiotics and antifungals. Labs were pertinent for normal chemistries, CBC, and negative infectious work-up including CMV PCR and Fungitell. Over the next 13 days, she developed worsening dyspnea and continued fevers. Further diagnostic evaluation and extensive infectious work-up were unrevealing. Chest imaging worsened and progressed to show bilateral hilar opacities and airspace disease. EBV quantitative PCR results at >750,000 copies and immunosuppression is stopped on POD 83. A transthoracic echo revealed RV failure secondary to vascular compression. Plans were made for initiation of VA ECMO and Rituximab, but she went into PEA arrest and expired on POD 89. Autopsy final pathology was consistent with donor derived diffuse large B cell lymphoma/monomorphic post-transplant lymphoproliferative disorder involving bilateral pulmonary hilum, mediastinum, and lung parenchyma.
This case illustrates a unique presentation of PTLD. It allows for discussion regarding the pathology and the rarity of donor derived PTLD in a patient who was EBV seropositive prior to transplantation. It also allows discussion regarding the unique timing (within 90 days of transplant) in this patient who received Alemtuzumab induction. Alemtuzumab causes a B-cell depletion, which can take 3-6 months to recover, and is therefore thought to decrease early PTLD.