The analysis of 165 children and adolescents/young adults with de novo blastic phase chronic myeloid leukemia showed disease status at hematopoietic stem cell transplantation was a strong prognostic ...factor and clearly separated the patient outcomes.
A new member of the UDP-
N-acetylglucosamine: β-galactose β1,3-
N-acetylglucosaminyltransferase (β3Gn-T) family having the β3-glycosyltransferase motifs was identified using an in silico method. This ...novel β3Gn-T was cloned from a human colon cancer cell line and named β3Gn-T8 based on its position in a phylogenetic tree and enzymatic activity. β3Gn-T8 transfers GlcNAc to the non-reducing terminus of the Galβ1–4GlcNAc of tetraantennary
N-glycan in vitro. HCT15 cells transfected with β3Gn-T8 cDNA showed an increase in reactivity to both LEA and PHA-L4 in a flow cytometric analysis. These results indicated that β3Gn-T8 is involved in the biosynthesis of poly-
N-acetyllactosamine chains on tetraantennary (β1,6-branched)
N-glycan. In most of the colorectal cancer tissues examined, the level of
β3Gn-T8 transcript was significantly higher than in normal tissue. β3Gn-T8 could be an enzyme involved in the synthesis of poly-
N-acetyllactosamine on β1–6 branched
N-glycans in colon cancer.
A 17-year-old Japanese man was referred to our hospital because of highly elevated serum levels of creatine kinase (CK) and transaminases. On admission, the proximal muscles of the lower extremities ...were found to be predominantly affected, and a score of 3/5 was obtained on Medical Research Council (MRC) scale. Muscular atrophy was evident and Gowers' sign was positive. His functional vital capacity (FVC) was markedly reduced. The results of the third edition of the Wechsler Adult Intelligence Scale (WAIS-III) indicated impairment of the patient's intelligence. Muscle biopsy showed scattered intracytoplasmic vacuoles with basophilic amorphous materials inside which were strongly stained by both periodic acid Schiff (PAS) and acid phosphatase. Biochemical analysis of the muscle tissue confirmed the diagnosis of GSDII because the glucosidase activity was 1.0 nmol/4 MU/mg/30 min (control range, 7.3 ± 2.2). Genetic analysis revealed a novel compound heterozygous missense mutation in GAA -c.1814 G >A (p.Gly605Asp) and c.1846 G >A (p.Asp616Asn) both in exon 13.
Solitary fibrous tumor (SFT) is a relatively uncommon mesenchymal tumor, and its occurrence in the meninges is rare. We herein report what is, to the best of our knowledge, the first cytological case ...of meningeal SFT metastatic to the lung with immunocytochemical analysis for signal transducer and activator of transcription 6 (STAT6), and compare the cytological characteristics to those of pleuropulmonary SFT. A 58-year-old Japanese male patient was found to have multiple nodules in the bilateral lungs after surgery for meningeal SFT. Partial resection of the nodules was performed, and touch smears were obtained. The Papanicolaou smear revealed cohesive hypercellular clusters of polygonal to elongated neoplastic cells with scant cytoplasm and oval to short spindle-shaped nuclei with nucleoli containing coarse chromatin. Mild-to-moderate nuclear pleomorphism was observed. No collagenous stroma was noted. Immunocytochemical analysis revealed that the neoplastic cells diffusely expressed STAT6. Histopathological and immunohistochemical studies confirmed the diagnosis of meningeal SFT metastatic to the lung. Moreover, reverse transcription-polymerase chain reaction analysis revealed that the lung tumor harbored
fusion. Recent studies demonstrated that there is a clinicopathological difference among
fusion variants in SFT. As reflected in fusion variants, meningeal SFT occasionally lacks collagenous stroma, as in the present case, although the most characteristic cytological feature of SFT is the presence of spindle-shaped neoplastic cells embedded in dense collagenous stroma. Thus, immunocytochemical analysis for STAT6 is very useful for diagnosing SFT.
Peripheral blood stem cell harvesting (PBSCH) is an established general practice; however, recent medical advances have led to diversification in hematopoietic stem cell transplantation and ...collection. A questionnaire survey on cell therapy and blood cell apheresis was conducted to understand the current situation and identify issues in Japan. A total of 159 facilities/departments responded to the survey. The annual number of autologous PBSCH was "11-25" in 48% and "1-10" in 37% of the centers, while the annual number of allogeneic PBSCH for related transplantation was "1-10" in 60% of the centers and "1-10" in 50% for unrelated transplantation. Collection sites were in the wards (40%), transfusion department (36%), and dialysis unit (26%). The apheresis system was staffed mainly by clinical engineers (67%) and doctors (23%). Primarily, doctors (96%) performed peripheral vascular punctures and were present at all times during collection in 37% of the centers. The Spectra Optia was the equipment most commonly used for apheresis. To freeze the product, 89% of the centers used "CP-1" without a controlled rate freezer. This survey revealed the diversification of collection methods in Japan, with "task shift" emerging as a significant concern.
A new member of the UDP-N-acetylglucosamine: beta-galactose beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T) family having the beta3-glycosyltransferase motifs was identified using an in silico ...method. This novel beta3Gn-T was cloned from a human colon cancer cell line and named beta3Gn-T8 based on its position in a phylogenetic tree and enzymatic activity. Beta3Gn-T8 transfers GlcNAc to the non-reducing terminus of the Galbeta1-4GlcNAc of tetraantennary N-glycan in vitro. HCT15 cells transfected with beta3Gn-T8 cDNA showed an increase in reactivity to both LEA and PHA-L4 in a flow cytometric analysis. These results indicated that beta3Gn-T8 is involved in the biosynthesis of poly-N-acetyllactosamine chains on tetraantennary (beta1,6-branched) N-glycan. In most of the colorectal cancer tissues examined, the level of beta3Gn-T8 transcript was significantly higher than in normal tissue. Beta3Gn-T8 could be an enzyme involved in the synthesis of poly-N-acetyllactosamine on beta1-6 branched N-glycans in colon cancer.
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