Fatal Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis with virus‐infected T cells after pediatric multivisceral transplantation: A proof‐of‐concept case report. (28th December 2020)

• Record Type: Journal Article
• Authors: Yamada, Masaki; Sakamoto, Seisuke; Sakamoto, Kenichi; Uchida, Hajime; Shimizu, Seiichi; Osumi, Tomoo; Kato, Motohiro; Shoji, Kensuke; Arai, Katsuhiro; Miyazaki, Osamu; Nakano, Noriyuki; Yoshioka, Takako; Fukuda, Akinari; Kasahara, Mureo; Imadome, Ken‐Ichi
• Is Part Of: Pediatric transplantation. Volume 25:Number 3(2021)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2020-12-28
• Subjects: Epstein‐Barr virus -- Hemophagocytic lymphohistiocytosis -- intestinal transplantation -- pediatrics; Transplantation of organs, tissues, etc. in children -- Periodicals; 617.95408305
• DOI: 10.1111/petr.13961
• Languages: English
• ISSNs: 1397-3142

Abstract

Abstract: Background: EBV‐associated HLH driven by EBV‐infected CD8 + T cells is a rare complication after pediatric solid organ transplantation. The etiology and disease spectrum of post‐transplant EBV‐HLH are poorly understood, and making a precise diagnosis and providing optimal treatment remain a challenge. Methods/Case description/Results: We report a 2‐year‐old multivisceral transplant recipient who developed fever and cytopenia with a persistent high EBV‐load state. Repeated tissue examinations and CT scans could not identify a localized mass, which is the key to the diagnosis of PTLD as per the WHO classification. Hence, EBV‐HLH was diagnosed by clinical manifestations as well as characterization of EBV‐infected cells, pathological examination on cell block of pleural effusion and clonality analysis. This EBV‐HLH did not respond to intensive chemotherapy, resulted in the recipient's death, acting similarly to hematological malignancy. Conclusions: Characterization of EBV‐infected cells in peripheral blood should be considered when persistent high EBV loads develop with symptoms consistent with PTLD, but no evidence of localized mass, and the tissue diagnosis is unavailable after pediatric solid organ transplantation.