Treatment for adult IgA vasculitis (IgAV), particularly severe cases, is not well established, although recent reports have suggested potential efficacy of rituximab (RTX). Here, we report a case highlighting the effectiveness of RTX based on pathophysiological and pathological considerations of IgAV, as well as a treatment protocol that has not been previously described. A 50-year-old man receiving glucocorticoids for cutaneous IgAV developed nephrotic syndrome and acute nephritic syndrome. Renal biopsy showed marked endocapillary and extracapillary proliferation with IgA deposition, consistent with severe IgAV nephritis. Despite glucocorticoid pulse therapy and intravenous cyclophosphamide, rapidly progressive glomerulonephritis ensued, prompting RTX initiation. With reference to peripheral blood CD19± B-cell monitoring, a single-dose RTX induction regimen was selected. Following RTX administration, the patient showed a favorable renal response. This case supports the potential efficacy of RTX in highly active IgAV with systemic symptoms. It suggests that a single-dose RTX induction approach may represent a feasible strategy to balance effective disease control with reduced infection risk in selected patients with severe adult IgAV, for which peripheral blood CD19+ B-cell monitoring may be helpful.
Rituximab-associated colitis, a rare toxicity that may occur following prolonged latency, has nonspecific features. We report a 42-year-old female with diffuse cutaneous systemic sclerosis and interstitial lung disease whose last rituximab infusion was 195 days before admission. She developed diarrhea and abdominal pain; initial computed tomography revealed no intra-abdominal focus. On day 12, colonoscopy revealed erosions and ulcers from the cecum to the descending colon; cytomegalovirus testing was negative. Symptoms persisted; repeat colonoscopy on day 20 confirmed multifocal ulceration, suggesting cytomegalovirus colitis or severe Crohn’s disease. By day 27, computed tomography showed ascending colon thinning, descending colon thickening, and haustral loss like ulcerative colitis. High-dose prednisolone was initiated owing to the inflammatory bowel disease-like process. Although clinical symptoms and inflammatory markers were improved, follow-up computed tomography revealed free air and perforation, necessitating emergency subtotal colectomy with ileostomy. Histology demonstrated ulcers extending to the subserosa, and noncaseating granulomas in the mucosa and submucosa. Immunohistochemistry demonstrated CD3-positive, CD20-negative, and CD4-predominant lymphocytic infiltration. This case highlights that rituximab-associated colitis may mimic inflammatory bowel disease, show prolonged latency, and progress to perforation despite apparent biochemical improvement, warranting early biopsy and careful radiologic follow-up. Rituximab-associated colitis should be considered even months following anti-CD20 exposure.
Primary hepatic undifferentiated carcinoma is an extremely rare and aggressive malignancy with no established systemic therapy, and the efficacy of immune checkpoint inhibitors (ICIs) remains unknown. We report a 67-year-old man with compensated hepatitis C–related cirrhosis who presented with a 27 mm segment 8 liver lesion and lymph node and bone metastases. Liver biopsy revealed undifferentiated carcinoma with high programmed death-ligand 1 (PD-L1) expression, and combination therapy with durvalumab and tremelimumab (Dur/Tre) was initiated. Shortly after treatment, the patient developed cranial nerve palsies (III, VII, VIII), suspected to represent either an immune-related adverse event or Ramsay Hunt syndrome; his symptoms improved with prednisolone and valaciclovir, allowing Dur/Tre to be resumed. After four courses, partial tumor shrinkage was achieved, and after 17 courses the primary tumor had decreased to 10 mm with complete resolution of nodal metastases, without further significant adverse events. To our knowledge, this is the first report of primary hepatic undifferentiated carcinoma with high PD-L1 expression demonstrating a sustained response to Dur/Tre. This case suggests that PD-L1 expression may serve as a potential biomarker for predicting ICI responsiveness in this rare and aggressive tumor.
Mid-pregnancy cervical length (CL) has limited predictive performance for spontaneous preterm birth (sPTB) in the normal range. The uterocervical angle (UCA) is a promising marker, but evidence regarding late-pregnancy UCA and its longitudinal change is limited. We evaluated UCA in mid- and late-pregnancy and the
Commercial radiochromic films, more specifically EBT-model Gafchromic films (EBT films), have the potential for two-dimensional profile measurements of ultra-high dose-rate (UHDR) radiation beams designed for FLASH radiotherapy. However, there is a controversy in regard to the dose-rate dependency of EBT-film responses to UHDR protons. In this study, we irradiated two types of currently available EBT films, EBT-XD and EBT4, to spread-out brag peak proton beams at 9 Gy at 180 Gy s-1 and 720 Gy s-1 in parallel and vertical beam incidences. The irradiated films were scanned with a flatbed scanner, and the inverted red color intensities were converted to absorbed doses based on the dose response curves of the red color component measured with LINAC 6 MV X-rays. The obtained dose levels were 5-15 % lower than the delivered doses, indicating the quenching effects along the tracks of high-LET charged particles. The depth dose profile obtained with the EBT-XD film at 720 Gy s-1 was notably higher than that at 180 Gy s-1, whereas such a difference was not observed with EBT4. The enhanced response of the EBT-XD film was confirmed by the sectional beam profiles obtained using vertically incident beams. These results imply that radiochromic reactions in EBT-XD film can be enhanced at the center of a Gaussian-shaped proton beam under certain UHDR conditions. Further studies are necessary to clarify the mechanism underlying the change in the responses of EBT films to UHDR protons.
