Late-Onset Posttransplant Lymphoproliferative Disorder (PTLD) in a Renal Transplant Patient

October 2013, Vol 144, No. 4_MeetingAbstracts Critical Care | October 2013

Late-Onset Posttransplant Lymphoproliferative Disorder (PTLD) in a Renal Transplant Patient Joshua Denson, MD; Abigail Maller, MD; Vikramjit Mukherjee, MD; Gregory Schrank, MD; Nancy Amoroso, MD; David Schwartz, MD New York University, New York, NY Chest. 2013;144(4_MeetingAbstracts):336A. doi:10.1378/chest.1705092

Abstract SESSION TITLE: Critical Care Student/Resident Case Report Posters II SESSION TYPE: Medical Student/Resident Case Report PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM INTRODUCTION: PTLD has an incidence of 1-5% in transplant recipients, with an average delay between transplantation and diagnosis of 14 months. More than 70% of cases occur within 5 years of transplant. We present a case of late onset PTLD developing in a patient 10 years after renal transplant. CASE PRESENTATION: A 43 year old woman with Down’s syndrome, on chronic immunosuppression since renal transplantation 10 years prior presented with non-exertional chest discomfort and lethargy. She was referred to the hospital by her primary physician for a 2 month history of an enlarging pericardial effusion. Exam was notable for fever, tachypnea, holosystolic murmur, and altered mental status. Laboratory studies revealed lymphopenia (300cells/uL). Transthoracic echocardiography revealed a pericardial effusion with tamponade physiology that drained 600ml of serosanguinous fluid. Lumbar puncture revealed an elevated opening pressure(38cm of H2O), elevated protein (613mg/dL), low glucose (29mg/dL), low pH (unrecordable) and elevated lactate (10.7 mmol/L). In contrast, arterial blood gas showed a pH of 7.6 with normal lactate. CSF EBV PCR was highly elevated at 1,600,000cpy/ml, whereas peripheral EBV PCR revealed only 2450cpy/ml. Brain MRI showed nodular and multifocal periventricular enhancement with obstructive hydrocephalus. Brain biopsy showed a monomorphic PTLD consistent with diffuse large B-cell lymphoma with plasmacytic differentiation, CD20 positive. The patient remained hemodynamically stable and was transferred to oncology to begin chemotherapy. DISCUSSION: Though PTLD is not uncommon in immunosuppressed patients after organ transplantation, it usually occurs within the first year or with an increase in immunosuppression. Our patient presented very late and atypically with pericardial tamponade and a frontal lobe lesion. Additionally, she had profound central neurogenic hyperventilation presumptively due to tumor related CSF lactate prompting “paradoxical” peripheral “compensatory” respiratory alkalosis.

CONCLUSIONS: Though PTLD most commonly develops soon after transplant or with increases in immunosuppression, a high index of suspicion should be maintained as long as immunosuppression continues. Our case also exemplifies classic central neurogenic hyperventilation related to tumor-associated CSF lactic acidosis. Reference #1: Naik S, Tayapongsak K, Robbins K, Manavi CK, Pettenati MJ, Grier DD. Burkitt's Lymphoma Presenting as Late-Onset Posttransplant Lymphoproliferative Disorder following Kidney and Pancreas Transplantation: Case Report and Review of the Literature. Case Rep Oncol. 2013 Jan;6(1):6-14. Reference #2: Gaviani P, Gonzalez RG, Zhu JJ, Batchelor TT, Henson JW. Central neurogenic hyperventilation and lactate production in brainstem glioma. Neurology 2005 Jan 11;64(1):1667. DISCLOSURE: The following authors have nothing to disclose: Joshua Denson, Abigail Maller, Vikramjit Mukherjee, Gregory Schrank, Nancy Amoroso, David Schwartz No Product/Research Disclosure Information