Posttransplant lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid or plasmacytic proliferations frequently\ndriven by Epstein-Barr virus (EBV). EBV-negative PTLDs appear to represent a distinct entity. This report describes an unusual\ncase of a 33-year-old woman that developed a monomorphic EBV-negative PTLD consistent with diffuse large B-cell lymphoma\n(DLBCL) 13 years after heart-lung transplant. Histological examination revealed marked pleomorphism of the malignant cells\nincluding nodular areas reminiscent of classical Hodgkin lymphoma (cHL) with abundant large, bizarre Hodgkin-like cells. By\nimmunostaining, themalignant cellswere immunoreactive for CD45, CD20, CD79a, PAX5, BCL6,MUM1, and p53 and negative for\nCD15, CD30, latentmembrane protein 1 (LMP1), and EBV-encodedRNA(EBER). Flowcytometry demonstrated lambda light chain\nrestricted CD5 and CD10 negative B-cells. Fluorescence in situ hybridization studies (FISH) were negative for cMYC, BCL2, and\nBCL6 rearrangements but showed deletion of TP53 and monosomy of chromosome 17. Next-generation sequencing studies (NGS)\nrevealed numerous genetic alterations including 6 pathogenicmutations in ASXL1, BCOR, CDKN2A, NF1, and TP53(x2) genes and\n30 variants of unknown significance (VOUS) in ABL1, ASXL1, ATM, BCOR, BCORL1, BRNIP3, CDH2, CDKN2A, DNMT3A, ETV6,\nEZH2, FBXW7, KIT, NF1, RUNX1, SETPB1, SF1, SMC1A, STAG2, TET2, TP53, and U2AF2.
Loading....