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BK polyomavirus-associated hemorrhagic cystitis

Background

Overview

Definition
BKPyV-HC is a hemorrhagic inflammation of the bladder caused by the BKPyV.
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Pathophysiology
The pathophysiology of BKPyV-HC involves the reactivation of BKPyV, which primarily targets the epithelial cells of the bladder. This reactivation is particularly common in immunocompromised individuals, such as those who have undergone organ or stem cell transplantation.
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Epidemiology
The incidence of BKPyV-HC is estimated at 27% in children and adolescents undergoing hematopoietic cell transplantation.
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Risk factors
Risk factors for BKPyV-HC include immunosuppression, organ transplantation, and allogeneic HSCT.
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Disease course
Clinically, BKPyV-HC presents with dysuria, lower abdominal pain, and hematuria. The severity of hematuria is often graded, with grade ≥ 2 being a key diagnostic criterion.
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Prognosis and risk of recurrence
The prognosis of BKPyV-HC varies. While the disease is typically self-limiting in immunocompetent individuals, it can lead to severe complications, including urinary obstruction and renal dysfunction, in immunocompromised patients.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of BK polyomavirus-associated hemorrhagic cystitis are prepared by our editorial team based on guidelines from the European Conference on Infections in Leukaemia (ECIL 2018) and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2009).
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Screening and diagnosis

Indications for screening: as per ECIL 2018 guidelines, do not obtain screening for BKPyV-HC in asymptomatic patients at risk following HSCT, as pre-emptive therapy is not established.
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  • Diagnostic criteria

Respiratory support

Hyperbaric oxygen therapy: as per ECIL 2018 guidelines, consider offering hyperbaric oxygen therapy aimed at speeding the healing process of the damaged urothelial lining in patients with BKPyV-HC following HSCT.
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Medical management

Reduction of immunosuppression: as per ECIL 2018 guidelines, insufficient evidence to provide the risk/benefit ratio of reduction of immunosuppression in patients with BKPyV-HC following HSCT. Balance the reduction of immunosuppression with the risk of worsening or triggering acute GvHD.
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  • Supportive therapy

  • Antiviral therapy

  • Agents with no evidence for benefit

Therapeutic procedures

Fibrin glue: as per ECIL 2018 guidelines, consider applying fibrin glue aimed at speeding the healing process of the damaged urothelial lining in patients with BKPyV-HC following HSCT.
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Preventative measures

Prophylaxis
As per ECIL 2018 guidelines:
Consider offering hyperhydration
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and performing bladder irrigation for BKPyV-HC prophylaxis aiming to reduce urothelial damage, particularly when using myeloablative conditioning based on cyclophosphamide, busulfan and total body irradiation.
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Recognize that specific antiviral prophylaxis is not available. Do not use fluoroquinolones given the lack of significant effects on BKPyV replication and hemorrhagic cystitis severity, and the selection of antibiotic resistance.
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