Polyomavirus Activation in Pediatric Patients with Hemorrhagic Cystitis Following Hematopoietic Stem Cell Transplantation / 임상소아혈액종양

BACKGROUND: Reactivation of the polyomavirus and the use of conditioning regimen may be the causes of hemorrhagic cystitis (HC) following hematopoietic stem cell transplantation (HSCT). However, there are only a few reports on the clinical characteristics of viral reactivation in HC following HSCT in Korea, especially in pediatric population.METHODS: 51 patients who received HSCT in Ajou University Hospital from January 2006 to June 2012 were investigated retrospectively. 16 patients were diagnosed with HC following HSCT and were enrolled in this study. Confirmation of polyomavirus was done by polymerase chain reaction (PCR) method.RESULTS: Out of the 16 patients diagnosed with HC following HSCT, there were 5 early type HC patients and 11 late type HC patients. Positive PCR results for the BK virus (BKV) and the JC virus were found on 13 and 5 patients, respectively. 4 patients showed positive results for both viruses. For the late type HC, there were 10 patients with positive PCR results for the BKV. Cyclophosphamide was used in 33 patients, and 13 patients eventually developed HC. There was no statistical significance between the incidence of hematuria and the reactivation of the BKV or the conditioning regimens. Most patients were treated conservatively but 4 patients who showed severe hematuria or poor general condition received intravenous cidofovir. After the infusion of cidofovir, hematuria disappeared on average of 65 days and the BKV was undetectable on average of 53 days.CONCLUSION: In our study, activation of the BKV was common in patients who were diagnosed with HC following HSCT. All patients recovered from HC with conservative management and the BKV became undetectable in the majority of patients who were treated with intravenous cidofovir.

Diffuse Large B Cell Lymphoma Associated with Epstein-Barr Virus Infection in a Liver Transplanted Child / 임상소아혈액종양

We report a case of 13 years old male patient. He was diagnosed as glycogen storage disease Ib at 6 month age, and received cadaver donor liver transplantation at 2 years and 4 months. He underwent immunosuppression for 9 years with several immunosuppressants including tacrolimus. Then he visited hospital for gum swelling and showed multiple malignancy suspicious lesions at nasal cavity, maxilla, mandibula, frontal bone, temporal bone, C1 and C2 spines, and several submandibular lymph nodes at radiologic study. Biopsy was done at oral cavity lesion, and histologically diagnosed as diffuse large B-cell lymphoma. The tissue specimen showed positivity in Epstein-Barr virus polymerase chain reaction (PCR). After diagnosis, the patient stopped all immunosuppressive agents and received 9 cycles of CHOP (cyclophosphamide, adriamycin, vincristine and prenisolone) chemotherapy for 8 months, then the patient achieved radiologic remission state, and being well without any signs of recurrence for two years of follow up.

Polyomavirus Activation in Pediatric Patients with Hemorrhagic Cystitis Following Hematopoietic Stem Cell Transplantation / 임상소아혈액종양

BACKGROUND: Reactivation of the polyomavirus and the use of conditioning regimen may be the causes of hemorrhagic cystitis (HC) following hematopoietic stem cell transplantation (HSCT). However, there are only a few reports on the clinical characteristics of viral reactivation in HC following HSCT in Korea, especially in pediatric population. METHODS: 51 patients who received HSCT in Ajou University Hospital from January 2006 to June 2012 were investigated retrospectively. 16 patients were diagnosed with HC following HSCT and were enrolled in this study. Confirmation of polyomavirus was done by polymerase chain reaction (PCR) method. RESULTS: Out of the 16 patients diagnosed with HC following HSCT, there were 5 early type HC patients and 11 late type HC patients. Positive PCR results for the BK virus (BKV) and the JC virus were found on 13 and 5 patients, respectively. 4 patients showed positive results for both viruses. For the late type HC, there were 10 patients with positive PCR results for the BKV. Cyclophosphamide was used in 33 patients, and 13 patients eventually developed HC. There was no statistical significance between the incidence of hematuria and the reactivation of the BKV or the conditioning regimens. Most patients were treated conservatively but 4 patients who showed severe hematuria or poor general condition received intravenous cidofovir. After the infusion of cidofovir, hematuria disappeared on average of 65 days and the BKV was undetectable on average of 53 days. CONCLUSION: In our study, activation of the BKV was common in patients who were diagnosed with HC following HSCT. All patients recovered from HC with conservative management and the BKV became undetectable in the majority of patients who were treated with intravenous cidofovir.

Diffuse Large B Cell Lymphoma Associated with Epstein-Barr Virus Infection in a Liver Transplanted Child / 임상소아혈액종양

We report a case of 13 years old male patient. He was diagnosed as glycogen storage disease Ib at 6 month age, and received cadaver donor liver transplantation at 2 years and 4 months. He underwent immunosuppression for 9 years with several immunosuppressants including tacrolimus. Then he visited hospital for gum swelling and showed multiple malignancy suspicious lesions at nasal cavity, maxilla, mandibula, frontal bone, temporal bone, C1 and C2 spines, and several submandibular lymph nodes at radiologic study. Biopsy was done at oral cavity lesion, and histologically diagnosed as diffuse large B-cell lymphoma. The tissue specimen showed positivity in Epstein-Barr virus polymerase chain reaction (PCR). After diagnosis, the patient stopped all immunosuppressive agents and received 9 cycles of CHOP (cyclophosphamide, adriamycin, vincristine and prenisolone) chemotherapy for 8 months, then the patient achieved radiologic remission state, and being well without any signs of recurrence for two years of follow up.