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Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease.

Abstract

Vulvovaginal graft-versus-host disease (GVHD) is an underdiagnosed and poorly recognized complication of hematopoietic stem cell transplantation (HSCT). Previous studies have reported findings restricted to predominantly adult populations. We report a case series of pediatric and young adult vulvovaginal GVHD, which was identified in 19 patients (median age, 11.8 years; range, 2.4 to 21.9 years) out of a total 302 female patients who underwent transplantation over an 8-year period at a pediatric HSCT center. The majority of patients had concomitant nongenital GVHD; only 1 patient had isolated vulvovaginal GVHD. The median time from bone marrow transplantation to diagnosis of vulvovaginal GVHD was 30 months (range, 2.3 to 97.5 months). A high percentage of the patients in our series were without vulvar or vaginal symptoms (n = 8; 42%), even though 17 patients (89%) presented with grade 3 disease based on current adult grading scales. Vulvar examination findings most frequently included interlabial and clitoral hood adhesions (89%), loss of architecture of the labia minora or clitoral hood (42%), and skin erosions or fissures (37%). Only 5 patients underwent a speculum exam, none of whom had vaginal GVHD. Examination findings of primary ovarian insufficiency (POI) can overlap with those of GVHD, and 6 patients (32%) in our cohort were diagnosed with POI. Only 1 patient was on systemic hormone replacement therapy at the time of vulvovaginal GVHD diagnosis. The majority of patients (n = 16) were treated with topical steroid therapy, with a median time to response of 43 days. Five patients (26%) had a complete response to therapy, and 10 patients (53%) had a partial response. This case series provides valuable insight into pediatric and young adult vulvovaginal GVHD and highlights the need for increased screening for vulvar disease in this population.

Authors+Show Affiliations

Division of Pediatric and Adolescent Gynecology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: Stephanie.cizek@gmail.com.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.Division of Pediatric and Adolescent Gynecology, Washington University School of Medicine, St Louis, Missouri.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31325588

Citation

Cizek, Stephanie M., et al. "Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease." Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation, 2019.
Cizek SM, El-Bietar J, Rubinstein J, et al. Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease. Biol Blood Marrow Transplant. 2019.
Cizek, S. M., El-Bietar, J., Rubinstein, J., Dandoy, C., Wallace, G. H., Nelson, A., ... Hoefgen, H. R. (2019). Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease. Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation, doi:10.1016/j.bbmt.2019.07.015.
Cizek SM, et al. Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease. Biol Blood Marrow Transplant. 2019 Jul 17; PubMed PMID: 31325588.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pediatric and Young Adult Vulvovaginal Graft-versus-Host Disease. AU - Cizek,Stephanie M, AU - El-Bietar,Javier, AU - Rubinstein,Jeremy, AU - Dandoy,Christopher, AU - Wallace,Gregory H, AU - Nelson,Adam, AU - Khandelwal,Pooja, AU - Myers,Kasiani C, AU - Hoefgen,Holly R, Y1 - 2019/07/17/ PY - 2019/04/25/received PY - 2019/07/11/revised PY - 2019/07/11/accepted PY - 2019/7/22/pubmed PY - 2019/7/22/medline PY - 2019/7/21/entrez KW - GVHD KW - Gynecology KW - Pediatric KW - Survivorship KW - Vaginal KW - Vulvar JF - Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation JO - Biol. Blood Marrow Transplant. N2 - Vulvovaginal graft-versus-host disease (GVHD) is an underdiagnosed and poorly recognized complication of hematopoietic stem cell transplantation (HSCT). Previous studies have reported findings restricted to predominantly adult populations. We report a case series of pediatric and young adult vulvovaginal GVHD, which was identified in 19 patients (median age, 11.8 years; range, 2.4 to 21.9 years) out of a total 302 female patients who underwent transplantation over an 8-year period at a pediatric HSCT center. The majority of patients had concomitant nongenital GVHD; only 1 patient had isolated vulvovaginal GVHD. The median time from bone marrow transplantation to diagnosis of vulvovaginal GVHD was 30 months (range, 2.3 to 97.5 months). A high percentage of the patients in our series were without vulvar or vaginal symptoms (n = 8; 42%), even though 17 patients (89%) presented with grade 3 disease based on current adult grading scales. Vulvar examination findings most frequently included interlabial and clitoral hood adhesions (89%), loss of architecture of the labia minora or clitoral hood (42%), and skin erosions or fissures (37%). Only 5 patients underwent a speculum exam, none of whom had vaginal GVHD. Examination findings of primary ovarian insufficiency (POI) can overlap with those of GVHD, and 6 patients (32%) in our cohort were diagnosed with POI. Only 1 patient was on systemic hormone replacement therapy at the time of vulvovaginal GVHD diagnosis. The majority of patients (n = 16) were treated with topical steroid therapy, with a median time to response of 43 days. Five patients (26%) had a complete response to therapy, and 10 patients (53%) had a partial response. This case series provides valuable insight into pediatric and young adult vulvovaginal GVHD and highlights the need for increased screening for vulvar disease in this population. SN - 1523-6536 UR - https://www.unboundmedicine.com/medline/citation/31325588/Pediatric_and_Young_Adult_Vulvovaginal_Graft-versus-Host_Disease L2 - https://linkinghub.elsevier.com/retrieve/pii/S1083-8791(19)30447-1 DB - PRIME DP - Unbound Medicine ER -