Vaginal/Vulvar Graft Versus Host Disease in Patients Transplanted in a Pediatric Hematopoietic Stem Cell Transplant Center

Background: Vaginal/vulvar graft versus host disease (vGVHD) is an under-diagnosed and poorly recognized complication of hematopoietic stem cell transplant (HSCT). Previous single-institution studies have reported findings restricted to predominantly adult populations. There exists no previous data reporting experience with this entity from a pediatric transplant program. We present our data of known cases of vGVHD observed at our center. Methods: Seventeen known cases of vGVHD from HSCT patients transplanted over eight years (2009-2017) were reviewed. We present these patientsÕ symptoms, signs, treatment and response. A detailed review of all of the HSCT clinical data was performed including diagnoses, transplant preparative regimen, stem cell source, GVHD prophylaxis and incidence. GVHD grading is based on standard Glucksberg criteria. Data is presented at times using medians and ranges. Results: Patient data including details of their HSCT are documented in Table 1. Ten of 17 patients actively complained of symptoms of concern including dysuria, vaginal irritation/burning, dyspareunia or discharge. Fourteen of 17 patients had significant findings including skin breakdown, labia majora or minora adhesions, or agglutination of the clitoral hood. Fourteen of 17 patients received topical steroid therapies with 6/14 requiring an increase in topical steroid potency for suboptimal response. One received only topical estrogen creams. Six patients have enjoyed a complete response to therapy while the remaining patients have either partial response or continue to have ongoing symptoms and/or signs to date. Twelve patients were receiving concurrent systemic immunosuppression at the time of vGVHD diagnosis.Table 1Vaginal/vulvar GVHD Patient DataCharacteristicNumberTotal patients17Median age (years) at time of HSCT12.8 (2.4-21.9)Median age (years) at time of vGVHD diagnosis14.1 (3.2-23)Median day post BMT of vGVHD diagnosis+593 (71-2253)Patients with Grade 1-4 acute skin GVHD8†Patients with chronic GVHD16Number of patients with symptoms (e.g. irritation, dysuria)10Median number of days between aGVHD and vGVHD diagnosis244 (39-652)Median number of days between cGVHD and vGVHD diagnosis92 (0-2160)Severity of vGVHD* Grade 11 Grade 21 Grade 314 unavailable1*Based on severity scales presented by Zantomio D et al. BMT 2006. Hirsch P et al. Transplantation 2012. Spinelli S et al. Haematologica 2003. Open table in a new tab *Based on severity scales presented by Zantomio D et al. BMT 2006. Hirsch P et al. Transplantation 2012. Spinelli S et al. Haematologica 2003. Conclusions: Female pediatric HSCT patients with a history of acute and/or chronic GVHD should receive frequent screening for vGVHD starting 100 days post-transplant. Current topical therapies oftentimes do not work and require intensification. We are currently developing a prospective pilot trial optimizing vGVHD screening and treatment to decipher the true incidence of the entity and the best strategies to resolve it.