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Abstract: SA-PO1117

BK Polyomavirus Nephropathy with Multiorgan Involvement: Whole-Genome Sequencing Data from a Killer Virus

Session Information

Category: Trainee Case Report

  • 1602 Pathology and Lab Medicine: Clinical

Authors

  • Roy, Sanjeet, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Weida, Carol J., Carolinas Pathology Group, Charlotte, North Carolina, United States
  • Huo, Jeffrey S., Levine Children''s Hospital, Charlotte, North Carolina, United States
  • Roehrs, Philip, Atrium Health, Levine Children's Hospital, Charlotte, North Carolina, United States
  • Grochowski, Darci L., Atrium Health, Charlotte, North Carolina, United States
  • Nalwa, Aasma, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Singh, Harsharan Kaur, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Mieczkowski, Piotr A., The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Nickeleit, Volker, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
Introduction

Post kidney transplantation “organ limited” BK-polyomavirus (BKPyV) nephropathy (BKN) is a known complication. BKPyV infections with multi-organ involvement and severe morbidity are rare. 6/10 reported cases occurred in patients with lymphoproliferative disorders, with AIDS (3/10) and post renal transplantation (1/10). Whether severe immunosuppression, specific BKPyV strains, and/or viral gene mutations promote systemic viral spread is unknown. Here we report the first case of fatal BKN with multi-organ involvement from which detailed deep virus genome sequencing data are available.

Case Description

Patient: 24-year old woman with sickle cell anemia, status post CD34+ selected, T-cell depleted allogeneic peripheral blood stem cell transplantation (tx). Clinical data: Multiple post tx episodes of GVHD. Since post tx day 174 progressive BKPyViremia, very low CD3, CD4, CD8 counts, low immunoglobulin titers. Since day 459 progressive renal failure. Since day 631 respiratory distress; no hemorrhagic cystitis. Since day 651 pancreatitis. On day 696 patient death due to renal and respiratory failure.
Autopsy findings: Productive PyV infection of both kidneys (PVN class 3), pancreas, and lungs with diffuse alveolar haemorrhage. Severe depletion of bone marrow and lymphoid organs.
Whole Genome Sequencing data (from kidney, lung and pancreas): No diagnostic genomic mutations; systemic infection by episomal BKPyV strain Ib2. Viral mutations restricted to NCCR control domain with severe deletions, duplications and insertions in the “P,Q,R” NCCR sequences (largely sparing the “O” and “S” sequences). BKPyV-NCCR mutations more abundant and profound than those reported in BKN. No genetic evidence of mutant BKPyV ‘metastatic’ spread from one organ site to another.

Discussion

This is the first report identifying the common Ib2 strain of BKPyV in a case of multi-organ infection. Presumably profound immunosuppression allowed for evolution of mutated BKPyV strains. Severe rearrangement solely observed in the viral control region NCCR likely enhanced replication and pathogenicity with infection of uncommon organs such as pancreas and lung. Renal failure and multi-organ involvement due to mutated BKPyV should be considered in severely immuno-compromised patients.