RIC HCT resolves DNA repair disorder-associated immunodeficiency. Long-term follow-up is required for secondary malignancy surveillance. Routine HCT for AT is not recommended.
The HPV-16/18 AS04-adjuvanted vaccine showed excellent prophylactic efficacy against 6-month persistent infection with HPV-16/18. The HPV-16/18 AS04-adjuvanted vaccine was generally well tolerated and immunogenic in the study population of healthy Japanese women aged 20 to 25 years.
Oncostatin M belongs to the interleukin-6 family of cytokines and acts as a multifunctional cytokine during murine embryogenesis and in inflammatory reactions. Although it has been demonstrated that oncostatin M has biological activities on many types of cells, including hepatocytes, dermal fibroblasts and endothelial cells, the roles of oncostatin M in the murine peripheral nervous system remain unclear. Here, we investigated the expression of specific beta-subunit of oncostatin M receptor in the dorsal root ganglia of adult mice. In the adult dorsal root ganglia, beta-subunit of oncostatin M receptor was exclusively expressed in small-sized neurons. Approximately 13% of total dorsal root ganglia neurons in mice contained beta-subunit of oncostatin M receptor. The double-immunofluorescence method revealed that approximately 28% of beta-subunit of oncostatin M receptor-positive neurons contained TrkA immunoreactivity, 63% expressed Ret immunoreactivity and 58% bound isolectin B4. No neuropeptides, including substance P and calcitonin gene-related peptide, were contained in the neurons. In addition, all beta-subunit of oncostatin M receptor-positive neurons expressed both vanilloid receptor 1 and P2X3 purinergic receptor. These neurons projected to the inner portion of lamina II in the dorsal horn of spinal cord and the dermis of skin. Seven days after sciatic nerve axotomy, the expression of beta-subunit of oncostatin M receptor was down-regulated in the lumbar dorsal root ganglia of the injured side. Our study demonstrated that beta-subunit of oncostatin M receptor was expressed in both cell bodies and processes of nonpeptidergic nociceptive neurons in adult mice, suggesting that oncostatin M may affect the nociceptive function of the neurons through the modulation of vanilloid receptor 1 and P2X3 expression.
A phase II, double-blind, controlled randomized multicenter study with human papillomavirus (HPV) 16/18 AS04 (3-O-desacyl-4'-monophosphoryl lipid A and aluminum hydroxide)-adjuvanted vaccine is ongoing in Japanese women aged 20 to 25 years. An interim analysis was performed at month 7 (1 month after the third dose of vaccine) to determine reactogenicity, safety, and immunogenicity of the vaccine and to evaluate the baseline HPV-16/18 seropositivity and DNA status of women. In the HPV-16/18 group (according-to-protocol cohort for immunogenicity analysis), 100% seroconversion was observed against HPV-16 and HPV-18 at month 6 (5 months after the second dose) and at month 7. At month 7, anti-HPV-16 geometric mean titer (GMT) was 7441.0 enzyme-linked immunosorbent assay units/mL and anti-HPV-18 GMT was 3805.4 enzyme-linked immunosorbent assay units/mL, which is, respectively, 250- and 168-fold higher than GMTs observed after natural infection with HPV-16 or HPV-18. In the total vaccinated cohort, the seropositivity rates against HPV-16 and HPV-18 at study entry were 17.3% and 15.8%, respectively. At the same time point, HPV-16 and HPV-18 DNA was detected in 6.5% and 4.0% of the women, respectively. The immunogenicity of the HPV-16/18 vaccine and the HPV prevalence before vaccination in Japanese women are in line with what was observed in other populations. Injection site symptoms and some general symptoms were reported more frequently in the HPV-16/18 group than in the hepatitis A vaccine group but had no impact on compliance with completion of the vaccination course. Overall, the HPV-16/18 vaccine had a good safety profile, was well tolerated, and is highly immunogenic in the study population of Japanese women.
The characteristics of adult patients with chronic active Epstein-Barr virus infection are poorly recognized, hindering early diagnosis and an improved prognosis. We studied 54 patients with adult-onset chronic active Epstein-Barr virus infection diagnosed between 2005 and 2015. Adult onset was defined as an estimated age of onset of 15 years or older. To characterize the clinical features of these adults, we compared them to those of 75 pediatric cases (estimated age of onset <15 years). We compared the prognosis of adult-onset chronic active Epstein-Barr virus infection with that of patients with nasal-type (n=37) and non-nasal-type (n=45) extranodal NK/T-cell lymphoma. The median estimated age of onset of these lymphomas was 39 years (range, 16–86 years). Compared to patients with pediatric-onset disease, those in whom the chronic active Epstein-Barr virus infection developed in adulthood had a significantly decreased incidence of fever (P=0.005), but greater frequency of skin lesions (P<0.001). Moreover, hypersensitivity to mosquito bites and the occurrence of hydroa vacciniforme were less frequent in patients with adult-onset disease (P<0.001 and P=0.0238, respectively). Thrombocytopenia, high Epstein-Barr virus nuclear antigen antibody titer, and the presence of hemophagocytic syndrome were associated with a poor prognosis (P=0.0087, P=0.0236, and P=0.0149, respectively). Allogeneic hematopoietic stem cell transplantation may improve survival (P=0.0289). Compared to pediatric-onset chronic active Epstein-Barr virus infection and extranodal NK/T-cell lymphoma, adult-onset chronic active Epstein-Barr virus infection had a poorer prognosis (P<0.001 and P=0.0484, respectively). Chronic active Epstein-Barr virus infection can develop in a wide age range, with clinical differences between adult-onset and pediatric-onset disease. Adult-onset chronic active Epstein-Barr virus infection is a disease with a poor prognosis. Further research will be needed.
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