A RARE CAUSE OF CRAZY PAVING: POSTTRANSPLANT LYMPHOPROLIFERATIVE DISEASE

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Crazy paving on computed tomography (CT) is defined as interlobular septal thickening with areas of ground glass attenuation. It can be caused by a variety of pathologies. Here we describe an unusual cause of crazy paving in an immunosuppressed patient. CASE PRESENTATION: A 58-year old man presented for syncope. His past medical history included acute myelogenous leukemia in complete remission post stem cell transplant, and complicated by cytomegalovirus colitis. Upon arrival to the emergency department, he was found to be obtunded and was intubated. Home medications included tacrolimus, ganciclovir, sulfamethoxazole-trimethoprim, and voriconazole. Vital signs were significant for a blood pressure of 62/38mmHg and heart rate of 138/minute. Laboratory studies revealed a white blood cell count of 13.6K/mm3, with 15% lymphocytes, 5% atypical lymphocytes, 34% plasmacytes, and 44% neutrophils. Lactic acid was 10.8mmol/L, bicarbonate 15mmol/L, and creatinine 3mg/dL. Arterial blood gas was consistent with metabolic acidosis. Tacrolimus level was 6ng/mL (goal 5-10). Computed tomography (CT) of the chest (Images 1-3) revealed interlobular septal thickening with ground glass opacities, and prominent mediastinal, cervical, and axillary lymphadenopathy. The patient required vasopressor support and was started on broad spectrum antibiotics, antivirals, antifungals. Bronchoalveolar lavage showed 38% neutrophils, 52% monocytes, and 10% lymphocytes. Gram stain, cultures, stain for acid fast bacilli, cytology, galactomannan, fungal cultures, and viral respiratory panel were negative. Serum flow cytometry revealed plasmacytoid cells with positive Epstein-Barr virus (EBV) in situ hybridization. Serum EBV PCR showed >39,000,000 copies/mL. These findings were consistent with post-transplant lymphoproliferative disease (PTLD). DISCUSSION: PTLD is a category of lymphoid disorders that arise after solid organ or hematopoietic transplants in the setting of active immunosuppression, most commonly with active EBV infection. Severity is graded as early, polymorphic, or monomorphic. Treatment can involve decreasing immunosuppression, rituximab in CD20+ disease, chemotherapy (with surgery if localized disease), or EBV-targeted cytotoxic T-cell instillation. Prevention of PTLD in transplant patients has included rapid tapering of immunosuppression as appropriate, and close monitoring of EBV viral levels, with elevations prompting reduction in immunosuppression or initiation of rituximab. Given its in vitro inhibition of EBV replication, ganciclovir has been suggested prophylactically, but there is no consensus regarding antiviral prophylaxis. CONCLUSIONS: Our patient rapidly decompensated and went into multiorgan system failure; he was made comfort care and expired. This case demonstrates the need for a high index of suspicion for PTLD in all transplanted patients with crazy paving present on imaging. Reference #1: Al-Mansour Z, Nelson BP, Evens AM. Post-transplant lymphoproliferative disease: risk factors, diagnosis, and current treatment strategies. Curr Hematol Malig Rep. 2013 September; 8(3): 173-183. Reference #2: Rossi SE, Erasmus JJ, Volpacchio M, et al. "Crazy Paving” pattern at thin-section CT of the lungs: radiologic-pathologic overview. Radiographics 2003; 26 (6): 1509-1519. Reference #3: AlDabbagh, MR, Gitman MR, Kumar D et al. The Role of Antiviral Prophylaxis for the prevention of Epstein-Barr virus-associated posttransplant lymphoproliferative disease in solic organ transplant recipients: a systematic review. Am J Transplant. 2017; 17: 770-781. DISCLOSURES: No relevant relationships by Payam Nabizadeh, source=Web Response No relevant relationships by Paul Simpson, source=Web Response