159 Post-Transplant Primary Effusion Lymphoma—Report of a Very Rare Case With Literature Review

Abstract

Post-transplant primary effusion lymphomas (PT-PEL) are extremely rare and often associated with fatal outcome. These are human herpesvirus 8 (HHV8)/Kaposi sarcoma herpesvirus (KSHV)-associated aggressive large B-cell lymphoma, typically presenting as serous effusions without detectable tumor masses. Kaposi sarcoma often precedes PT-PEL. PEL carries a very poor prognosis with a median survival time of less than six months. We report a recent such rare case of PT-PEL at our institution with concurrent Kaposi sarcoma in a cardiac transplant patient and present review of literature. The patient is a 63-year-old-man with congestive heart failure due to ischemic cardiomyopathy, who underwent cardiac transplantation five months ago. He was diagnosed with HHV8/KSHV-associated Kaposi sarcoma eight years ago and treated with chemoradiotherapy. He now presented with shortness of breath and left-sided exudative pleural effusion. ThinPrep slide and cell block revealed uniform single large atypical cells with high N:C ratio, coarsely granular chromatin and prominent nucleoli. The atypical tumor cells were positive for CD45, CD138, MUM1, CD30, and HHV8, while negative for CD20, Pax5, CD4, CD8, CD56, Alk-1, EBV, EMA, bcl2, bcl6, cyclin-D1, CD3, CD5, and CD10. Ki67 showed very high nuclear proliferation index (>95%). FISH showed immunoglobulin heavy chain (IgH) gene rearrangement. Combining all, the diagnosis of PT-PEL was made. Review of literature showed, PT-PEL is extremely rare. Since first described in 1998, only 12 cases have been reported, and only three of them were cardiac transplant recipients; two of these also had post-transplant Kaposi sarcoma. In contrast to reported cases, our case had HHV8/KSHV infection preceding the transplant with development of Kaposi sarcoma. PT-PEL is an extremely rare disease in reported literature. The case described here occurred in a patient with history of Kaposi sarcoma with subsequent cardiac transplantation, due to immune suppression with reactivation of HHV8/KSHV infection.