Posttransplant Lymphoproliferative Disorder Presenting as Cardiogenic Shock With Tamponade

Abstract

SESSION TITLE: Transplantation SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Post-transplant lymphoproliferative disorders (PTLD) are malignancies associated with solid organ transplantation or allogeneic hematopoietic cell transplantation requiring immunosuppression. Most cases are associated with EBV positivity, but less commonly EBV negative PTLD occurs. CASE PRESENTATION: A 55-year-old Caucasian male presented with cardiogenic shock due to pericardial effusion with tamponade. His history was significant for type I diabetes mellitus leading to ESRD, two failed renal transplants on immunosuppression, and current peritoneal dialysis dependence. Echocardiogram upon admission demonstrated pericardial effusion with right ventricular collapse. Pericardiocentesis and pericardial drain placement were performed. Approximately 2.4L of fluid was drained. Cytology of the cell block was negative for malignancy. He improved after drainage and was discharged on day 16. Two days after discharge he returned complaining of weakness. A left sided pleural effusion was detected and drained via catheter. Cell block cytology was remarkable for monomorphic PTLD, plasma cell neoplasm. Five days after pleural catheter placement he developed multifactorial shock that led to a whole-body CT with contrast. This revealed dilation of the jejunum measuring 5.1cm, as well as a mass that measured 8.4 x 5.6. x 5.4 cm. The mass demonstrated atypical plasma cells consistent with the cell block. Discontinuing immunosuppression was the initial step in treatment in this case. Further work up for plasmacytoma or multiple myeloma demonstrated negative SPEP and negative lambda ratio confirming a solitary plasmacytoma. A skeletal survey was completed with no signs of lytic lesions or evidence of multiple myeloma. A bone marrow biopsy was unable to be completed as the patient expired. DISCUSSION: The lesion consisted of atypical plasma cells similar to cases of EBV-negative PTLD previously mentioned in the literature. This is currently a designation not clearly defined by the World Health Organization. These findings call for further investigation to discover the mechanism of disease and promote effective therapy. Therapy in EBV-negative PTLD does not seem to respond to the established algorithm for EBV-positive PTLD. Reducing immunosuppression often makes no difference. Some cases require high dose immunosupression to achieve therapeutic success, which is the opposite of the standard treatment for PTLD. Defining the mechanism behind these tumors is imperative for better classification as well as directed therapy. CONCLUSIONS: This case is remarkable for PTLD with CD20 negativity; additionally, PCR for EBV was negative. EBV is typically the primary driver of PTLD. The mechanism of EBV negative PTLD remains unknown. Reference #1: Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117(19):5019-5032. doi:10.1182/blood-2011-01-293050. DISCLOSURE: The following authors have nothing to disclose: Matthew Boulos, Anil Singh, David Aljadir, Tina Dudney No Product/Research Disclosure Information