Page 1 - CUA Best Practice Report: Pediatric hemorrhagic cystitis

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CUA BEST PRACTICE REPORT

Canadian Urological Association Best Practice Report: Pediatric

hemorrhagic cystitis

Jessica H. Hannick, MD, MSc ; Martin A. Koyle, MD, MSc 2,3
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3
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1 Division of Pediatric Urology, UH Rainbow Babies and Children’s Hospital, Cleveland, OH, United States; The Hospital for Sick Children, Toronto, ON, Canada; Division of Urology, Department of Surgery,
University of Toronto, Toronto, ON, Canada
Cite as: Can Urol Assoc J 2019;13(11):E325-34. http://dx.doi.org/10.5489/cuaj.5993 tem for guideline recommendations, as employed by the
International Consultation on Urologic Disease (ICUD).

Published online March 29, 2019 Definition

HC is defined by the presence of hematuria and lower uri-
Introduction nary tract symptoms, such as dysuria, frequency, or urgen-
cy, in the absence of other potential contributing factors,
This best practice report aims to provide the general practic- such as vaginal bleeding or bacterial or fungal urinary tract
ing urologist with basic background information regarding infections. Multiple grading criteria have been published
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the pathophysiology and natural history of hemorrhagic cys- to distinguish the varied presentations of HC. Frequently
titis (HC) in the pediatric population, as well as diagnostic referenced grading schema are Droller and Arthur’s, which
and algorithmic therapeutic recommendations. Given that are used to aid the clinician in discerning potential treatment
2,3
HC in the pediatric population is most frequently diagnosed options and inform the clinician about prognosis (Table 1).
in the setting of bone marrow (BMT) or stem cell transplan- The European Organization for Research and Treatment of
tation (SCT), discussion and recommendations will focus Cancer has combined similar grading criteria, along with
largely on this population, but many of the diagnostic and quality of life parameters to further relay the morbidity and
treatment options can be expanded to broader pediatric mortality implications of each grade.
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populations affected by HC.
Background and natural history
Methodology
The reported incidence of HC ranges widely from 5‒70%
A systematic literature search was performed in the PubMed, irrespective of grade. Unlike in adults where radiation
5,6
Medline, and Cochrane Library databases using the term is the most common cause, the more frequent causes of
“hemorrhagic cystitis.” The search was limited to articles HC in children are the immediate and late effects of stem
from 1998 to the present day, with some exceptions made for cell and/or bone marrow transplantation for malignant and
seminal articles referenced in the bibliographies of multiple benign diseases. Rare reports of medications (risperidone)
manuscripts. Due to the paucity of high-quality systematic and other disease processes (ataxia telangiectasia) have also
reviews, meta-analyses, and large comparative studies writ- been linked to the development of HC. 7-9
ten on this topic, the majority of evidence and recommenda- Early-onset HC characterizes the immediate phase fol-
tions are based on lower-quality case series and case reports. lowing conditioning for transplantation, which carries no
The websites for the American Urological Association, the absolute definition but has been described as ranging any-
European Association of Urology, the Societies for Pediatric where from 48 hours to two weeks after conditioning. 6,10 The
Urology, the European Society for Pediatric Urology, the early-onset bleeding typically occurs as a result of either the
National Institute for Health Care and Excellence, the conditioning regimen itself — chemotherapy and/or radia-
Children’s Oncology Group, and the Société Internationale tion — and/or the intended myelosuppression and resultant
d’Oncologie Pédiatrique were examined for guidelines and thrombocytopenia. The chemotherapeutic agents most fre-
policies regarding the management of pediatric HC, but quently implicated in causing HC are the alkylating agents
none were available. — cyclophosphamide, ifosfamide, and busulfan — that yield
All manuscripts were reviewed using the modified acrolein as a hepatic metabolite, which results in bladder
Oxford Center for Evidence-Based Medicine grading sys- mucosal inflammation, sloughing, and thinning. 6,11,12 To

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