Post-renal Biopsy Retroperitoneal Haematoma Accompanied by Decreased Coagulation Factor XIII Levels in Immunoglobulin A Nephropathy.

Post-biopsy bleeding is the primary complication of renal biopsy. Retroperitoneal haematoma is a rare but severe bleeding complication; it commonly occurs among patients who have risk factors or vascular lesions. The bleeding risks in patients with immunoglobulin A (IgA) nephropathy (IgAN) have been discussed in the literature, but clinical data are lacking. Here, we report a case of a post-biopsy retroperitoneal haematoma accompanied by decreased coagulation factor XIII (FXIII) in a patient with IgAN. A 14-year-old male patient with haematuria and proteinuria but no bleeding or family history of bleeding underwent pre-renal biopsy evaluation that showed no coagulation abnormalities. He underwent percutaneous renal biopsy, and the histopathological diagnosis was IgAN. Five days after the biopsy, he presented with delayed bleeding from a retroperitoneal haematoma. During the workup for undiagnosed haemorrhagic diatheses, a mildly decreased FXIII level was discovered. This result suggested the possibility of bleeding complications associated with decreased FXIII. Some bleeding diatheses, including FXIII deficiency, cannot be evaluated in routine pre-biopsy coagulation tests. Mild FXIII deficiency can increase the risk of post-biopsy bleeding complications. Therefore, physicians should consider unevaluated haemorrhagic diatheses when a patient presents with major bleeding complications or delayed bleeding following renal biopsy without any known risk factors or vascular lesions.

Challenges to Widespread Use of Fertility Preservation Facilities for Pediatric Cancer Patients in Japan.

Purpose: Although fertility preservation for pediatric cancer patients is becoming more widespread in Japan, some facilities do not provide sufficient information regarding fertility. This study aimed to elucidate the problems pertaining to the lack of information about fertility among patients. Methods: Based on a 2020 survey, seminars addressing fertility preservation were held from the Designated Pediatric Cancer Care Hospitals in each of the seven blocks in Japan to their partner hospital (pediatric cancer hospitals). The seminar consisted of lectures and group discussions, and a questionnaire was also administered after each seminar. Results: In the group discussions, a lack of explanations to patients and explanatory materials for children were cited as issues by many facilities. The survey results revealed a lack of material explaining fertility preservation and a lack of knowledge among health care providers. There were also many requests to use the patient explanation videos presented at the seminar. Conclusion: The results indicate that further education for health care providers by seminar and other sources and enhancement of explanatory materials are important for fertility preservation in pediatric cancer hospitals in Japan.

Reduced-intensity allogenic transplantation for children and adolescents with Philadelphia chromosome-positive acute lymphoblastic leukemia.

Survival rates of patients with Philadelphia chromosome-positive ALL (Ph+ALL) have improved considerably with the introduction of tyrosine kinase inhibitors (TKI); however, hematopoietic stem cell transplantation (HSCT) continues to play an important role. Reduced-intensity conditioning (RIC) regimens have been widely applied particularly for older patients, but their validity for children and adolescents with Ph+ALL has not been investigated. In this study, data from patients receiving HSCT for de novo Ph+ALL in first or second remission at ages younger than 25 years and with a history of pre-HSCT TKI therapy were retrospectively collected through the nationwide registry in Japan. In 265 patients who received myeloablative conditioning (MAC) and 33 patients receiving RIC, 5-year leukemia-free survival (LFS) rates were 67.3% and 79.8%, respectively (p = 0.142). Multivariate analysis of LFS, focusing on patients with good performance status, identified RIC as a significant prognostic factor for LFS (hazard ratio 0.32, p = 0.032), as well as older age, higher leukocyte count at diagnosis, and disease with additional chromosomal abnormalities. These trends were similar when we focused on patients who received prophylactic post-HSCT TKI treatment, as 5-year LFS was 81.0% for MAC and 84.4% for RIC (p = 0.748). In summary, HSCT with RIC regimen showed at least comparable LFS to HSCT with MAC regimen, and RIC was an independent favorable prognostic factor on multivariate analysis adjusting potential prognostic factors. While patient numbers were limited, our data suggest that RIC may be safely applied in this group, particularly combined with prophylactic post-HSCT TKI maintenance therapy.