Intravascular large B-cell lymphoma(IVLBCL) is a rare and aggressive type of lymphoma with diverse and nonspecific clinical manifestations, often leading to misdiagnosis. This article reports a case of IVLBCL in a middle-aged male patient who initially presented with pulmonary arterial hypertension(PAH). The patient exhibited progressive hypoxemia and PAH, showing poor response to standard PAH therapy. Laboratory tests indicated a hyperinflammatory state and significantly elevated lactate dehydrogenase levels, while imaging revealed diffuse bilateral lung lesions. Random skin biopsy identified atypical B lymphocytes within subcutaneous capillaries, confirming the diagnosis of IVLBCL. Following treatment with the ZR-CHOP regimen, the patient's symptoms and laboratory parameters improved markedly. By reviewing relevant literature, this article systematically outlines the diagnostic and therapeutic process of this case, aiming to provide insights for the clinical recognition of such rare presentations.

Objective:To analyze the clinical characteristics of mannose phosphate isomerase-congenital disorders of glycosylation (MPI-CDG) and evaluated the outcomes following D-mannose treatment.Methods:This case-series study analyzed clinical manifestations, laboratory findings, imaging results, genetic data, and outcomes after D-mannose therapy in 5 children with MPI-CDG diagnosed at the Children′s Hospital of Fudan University between December 2014 and December 2024.Results:The age of onset ranged from 0.3 to 0.4 years in all 5 children, who initially presented with diarrhea and hypoglycemia. Associated manifestations included short stature (3 cases), anemia (3 cases), splenomegaly (3 cases), hepatomegaly (4 cases), elevated transaminases (4 cases), and hypoalbuminemia (4 cases). Liver pathology revealed hepatic fibrosis in 3 cases. Genetic testing identified 8 variants in the MPI gene, including 2 novel variants. Following D-mannose treatment, diarrhea and hypoglycemia resolved within 1-2 weeks in all children, with concurrent improvement in anemia. Notably except for Patient 1, who developed progressive splenomegaly, worsening hepatic fibrosis, and portal hypertension despite persistently normal transaminase and albumin levels, the other 4 children showed improvement in transaminase levels, resolution of hypoalbuminemia and amelioration of imaging abnormalities.Conclusions:MPI-CDG typically manifests in infancy with diarrhea and hypoglycemia, often accompanied by multi-system involvement. D-mannose treatment significantly improves metabolic abnormalities and most organ damages. However, close surveillance of liver status is warranted due to the risk of hepatic fibrosis progression in some cases.

The key points of the update of the content related to colorectal cancer and anal cancer in the Chinese Anti-Cancer Association (CACA) Guidelines for Integrative Oncology 2025 Edition (hereinafter referred to as the CACA 2025 Guidelines) include 4 aspects. In terms of epidemiology, the latest data on the incidence and mortality of colorectal cancer in China have been updated, and the recommended screening age has been adjusted. In diagnosis, the application of enhanced MRI examination in diagnosis has been optimized, and the recommendation for peripheral blood microsatellite instability (MSI) detection has been added. In terms of treatment, in surgical treatment, the total mesorectal excision of the right colon, the safety of the Natural Orifice Specimen Extraction Surgery (NOSES) technique, the applicable range of robotic surgery, and the high-level evidence-based medical evidence of transanal total mesorectal excision (taTME) have been newly added, and the principles of surgical treatment have been added as well. In medical treatment, the role of circulating tumor DNA (ctDNA) in treatment decision-making has been supplemented. The application of dual immunotherapy in advanced patients has been recommended, and the application of third-line and subsequent-line treatments in advanced patients has been newly added. The guidelines improved the principle of preoperative neoadjuvant radiotherapy for rectal cancer, changed the indication of short-course radiotherapy, and added a variety of chemoradiotherapy combinations and recommendations for the timing of surgery. In addition, the follow-up programs for colorectal cancer and anal canal cancer are clarified, and nutritional therapy, traditional Chinese medicine rehabilitation therapy and nursing care for sequelae are emphasized, which provide more scientific and comprehensive guidance for the diagnosis and treatment of colorectal cancer and anal canal cancer.

The key points of the update of the content related to colorectal cancer and anal cancer in the Chinese Anti-Cancer Association (CACA) Guidelines for Integrative Oncology 2025 Edition (hereinafter referred to as the CACA 2025 Guidelines) include 4 aspects. In terms of epidemiology, the latest data on the incidence and mortality of colorectal cancer in China have been updated, and the recommended screening age has been adjusted. In diagnosis, the application of enhanced MRI examination in diagnosis has been optimized, and the recommendation for peripheral blood microsatellite instability (MSI) detection has been added. In terms of treatment, in surgical treatment, the total mesorectal excision of the right colon, the safety of the Natural Orifice Specimen Extraction Surgery (NOSES) technique, the applicable range of robotic surgery, and the high-level evidence-based medical evidence of transanal total mesorectal excision (taTME) have been newly added, and the principles of surgical treatment have been added as well. In medical treatment, the role of circulating tumor DNA (ctDNA) in treatment decision-making has been supplemented. The application of dual immunotherapy in advanced patients has been recommended, and the application of third-line and subsequent-line treatments in advanced patients has been newly added. The guidelines improved the principle of preoperative neoadjuvant radiotherapy for rectal cancer, changed the indication of short-course radiotherapy, and added a variety of chemoradiotherapy combinations and recommendations for the timing of surgery. In addition, the follow-up programs for colorectal cancer and anal canal cancer are clarified, and nutritional therapy, traditional Chinese medicine rehabilitation therapy and nursing care for sequelae are emphasized, which provide more scientific and comprehensive guidance for the diagnosis and treatment of colorectal cancer and anal canal cancer.

Objective:To analyze the clinical characteristics of mannose phosphate isomerase-congenital disorders of glycosylation (MPI-CDG) and evaluated the outcomes following D-mannose treatment.Methods:This case-series study analyzed clinical manifestations, laboratory findings, imaging results, genetic data, and outcomes after D-mannose therapy in 5 children with MPI-CDG diagnosed at the Children′s Hospital of Fudan University between December 2014 and December 2024.Results:The age of onset ranged from 0.3 to 0.4 years in all 5 children, who initially presented with diarrhea and hypoglycemia. Associated manifestations included short stature (3 cases), anemia (3 cases), splenomegaly (3 cases), hepatomegaly (4 cases), elevated transaminases (4 cases), and hypoalbuminemia (4 cases). Liver pathology revealed hepatic fibrosis in 3 cases. Genetic testing identified 8 variants in the MPI gene, including 2 novel variants. Following D-mannose treatment, diarrhea and hypoglycemia resolved within 1-2 weeks in all children, with concurrent improvement in anemia. Notably except for Patient 1, who developed progressive splenomegaly, worsening hepatic fibrosis, and portal hypertension despite persistently normal transaminase and albumin levels, the other 4 children showed improvement in transaminase levels, resolution of hypoalbuminemia and amelioration of imaging abnormalities.Conclusions:MPI-CDG typically manifests in infancy with diarrhea and hypoglycemia, often accompanied by multi-system involvement. D-mannose treatment significantly improves metabolic abnormalities and most organ damages. However, close surveillance of liver status is warranted due to the risk of hepatic fibrosis progression in some cases.

Objective:To investigate the relationship between the polymorphism of endoplasmic reticulum aminopeptidase 1 (ERAP-1) gene and the occurrence of pre-eclampsia (PE).Methods:A case-control study was conducted in Beijing Obstetrics and Gynecology Hospital from October 2018 to October 2021. A total of 51 PE pregnant women with onset gestational age<34 weeks were selected as the PE group, and 48 normal pregnant women during the same period were selected as the control group. Venous blood samples were collected from the pregnant women before delivery and umbilical cord within 5 minutes after delivery. Single nucleotide polymorphisms (SNP) of ERAP-1 gene in the pregnant women and their fetus were detected by next-generation sequencing. Univariate analysis and multivariate logistic regression analysis were used to analyze all the SNP loci and alleles detected in the two groups, and the significant SNP were screened.Results:(1) A total of 13 target SNP loci of maternal ERAP-1 gene were selected by univariate analysis. Among them, the frequency distribution of genotypes at 96096828, 96121524, 96121715, 96122260 and 96122281 showed statistically significant differences between PE group and control group (all P<0.05). Multivariate logistic regression analysis showed that the risk of PE in pregnant women with TC genotype at locus 96121524 was 2.002 times higher than those with TT genotype (95% CI: 0.687-5.831, P=0.020). (2) A total of 4 target SNP loci of ERAP-1 gene in fetal were selected by univariate analysis, and there was no statistical significance in gene polymorphism of the 4 loci between PE group and control group (all P>0.05). Multivariate logistic regression analysis showed that the risk of PE in fetus with genotype AA at locus 96121406 was 0.236 times that of fetus with genotype GG (95% CI: 0.055-1.025, P=0.016). Conclusion:ERAP-1 gene with TC genotype at 96121524 in the mother and GG genotype at 96121406 in the fetus might be related to the incidence of PE.

Objective To explore the relationship between D-dimer and carcinoembryonic antigen (CEA) levels before treatment and clinicopathologic features of colorectal cancer and the value of prognosis. Methods A total of 209 patients who underwent radical colorectal cancer surgery in Wujin Hospital Affiliated of Jiangsu University from January 2017 to December 2018 were selected as the study objects. D-dimer and CEA levels were detected before treatment,and their relationship with clinicopathologic features and prognosis were analyzed. Results Before treatment,D-dimer was correlated with tumor site (P<0.001),pathological type (P=0.007),depth of invasion (P<0.001),lymph node metastasis (P=0.007) and tumor stage (P<0.001). CEA was associated with pathological type (P<0.001) and tumor stage (P=0.035). The postoperative tumor-free survival rate (x2=21.659,P<0.001) and overall survival rate (x2=22.887,P<0.001) in patients with both D-dimer and CEA positive expression before treatment were significantly lower than those in patients without both positive expression. The area under the curve for predicting overall survival of colorectal cancer patients with both D-dimer and CEA positive before treatment was 0.723. Pearson correlation analysis showed a positive correlation between D-dimer and CEA in colorectal cancer patients before treatment (r=0.144,P=0.037). Cox proportional risk regression analysis showed that simultaneous positive D-dimer and CEA,lymph node metastasis and distant metastasis were independent risk factors affecting postoperative tumor-free survival and overall survival of colorectal cancer patients (P<0.05). Conclusion D-dimer and CEA before treatment are of great value in the diagnosis and prognosis of colorectal cancer,and the combination of D-dimer and CEA has a definite effect on the accuracy of postoperative survival assessment of colorectal cancer.

Objective To explore the relationship between D-dimer and carcinoembryonic antigen (CEA) levels before treatment and clinicopathologic features of colorectal cancer and the value of prognosis. Methods A total of 209 patients who underwent radical colorectal cancer surgery in Wujin Hospital Affiliated of Jiangsu University from January 2017 to December 2018 were selected as the study objects. D-dimer and CEA levels were detected before treatment,and their relationship with clinicopathologic features and prognosis were analyzed. Results Before treatment,D-dimer was correlated with tumor site (P<0.001),pathological type (P=0.007),depth of invasion (P<0.001),lymph node metastasis (P=0.007) and tumor stage (P<0.001). CEA was associated with pathological type (P<0.001) and tumor stage (P=0.035). The postoperative tumor-free survival rate (x2=21.659,P<0.001) and overall survival rate (x2=22.887,P<0.001) in patients with both D-dimer and CEA positive expression before treatment were significantly lower than those in patients without both positive expression. The area under the curve for predicting overall survival of colorectal cancer patients with both D-dimer and CEA positive before treatment was 0.723. Pearson correlation analysis showed a positive correlation between D-dimer and CEA in colorectal cancer patients before treatment (r=0.144,P=0.037). Cox proportional risk regression analysis showed that simultaneous positive D-dimer and CEA,lymph node metastasis and distant metastasis were independent risk factors affecting postoperative tumor-free survival and overall survival of colorectal cancer patients (P<0.05). Conclusion D-dimer and CEA before treatment are of great value in the diagnosis and prognosis of colorectal cancer,and the combination of D-dimer and CEA has a definite effect on the accuracy of postoperative survival assessment of colorectal cancer.

AIM: To investigate the injury of emodin (EMO) in reduce of glomerular mesangial cells (MCs) in lupus nephritis by targeting forkhead protein K2 (FOXK2) through miR-96-5p. METHODS: The contents of 24 h urine protein, serum urea nitrogen (BUN) and serum creatinine (Scr) in MRL / faslpr mice (lupus nephritis group) and MRL / MPJ mice (control group) were detected. MCs were separated, purified and divided into: MCs group (MCs without any treatment), L-EMO group (MCs treated with 10 μmol/L Emodin), M-EMO group (MCs treated with 25 μmol / L Emodin), H-EMO group (MCs treated with 50 μmol / L Emodin), H-EMO + miR-96-5p-NC group (MCs treated with 50 μmol / L Emodin and transfected with miR-96-5p-NC), and H-EMO + miR-96-5p-minic group (MCs treated with 50 μmol/ L Emodin and transfected with miR-96-5p-minic). Double luciferase report experiment was used to verify the targeting relationship between miR-96-5p and FOXK2. The real-time quantitative fluorescent polymerase chain reaction (qRT-PCR) was used to detect the expression of miR-96-5p. Western blot was used to detect the expression of FOXK2 and apoptosis related proteins. The enzyme-linked immunosorbent assay (ELISA) was used to detect the levels of inflammatory factors in MCs. cell count kit 8 (CCK-8) was used to determine the activity of MCs. Annexin-V FITC/PI double staining was used to detect apoptosis of MCs. RESULTS: Compared with the control group, 24 h urinary protein content, serum BUN and Scr levels in the lupus nephritis group were significantly increased (P< 0.05). Compared with the MCs group, the miR-96-5p expression, interleukin1β (IL-1β), interleukin6 (IL-6), tumor necrosis factor-α (TNF-α), A450 value and B-lymphoblastoma-2 (Bcl-2) protein in the L-EMO group, M-EMO group and H-EMO group were significantly decreased (P<0.05), the FOXK2 level, cell apoptosis rate, Bcl-2 related X gene (Bax), aspartate specific cysteine proteinase-3 (cleaved Caspase-3) protein levels were significantly increased, respectively (P<0.05), the effect of Emodin was dose-dependent. Compared with the H-EMO group and H-EMO+miR-96-5p-NC group, H-EMO+miR-96-5p-minic group obviously increased the miR-96-5p expression, inflammatory factor levels, A450 value and Bcl-2 protein level (P<0.05), and obviously decreased FOXK2 level and cell apoptosis rate (P< 0.05). CONCLUSION: EMO can reduce the injury of lupus nephritis MCs by down-regulating miR-96-5p and then up-regulating FOXK2.

The progressive destruction of condylar cartilage is a hallmark of the temporomandibular joint (TMJ) osteoarthritis (OA); however, its mechanism is incompletely understood. Here, we show that Kindlin-2, a key focal adhesion protein, is strongly detected in cells of mandibular condylar cartilage in mice. We find that genetic ablation of Kindlin-2 in aggrecan-expressing condylar chondrocytes induces multiple spontaneous osteoarthritic lesions, including progressive cartilage loss and deformation, surface fissures, and ectopic cartilage and bone formation in TMJ. Kindlin-2 loss significantly downregulates the expression of aggrecan, Col2a1 and Proteoglycan 4 (Prg4), all anabolic extracellular matrix proteins, and promotes catabolic metabolism in TMJ cartilage by inducing expression of Runx2 and Mmp13 in condylar chondrocytes. Kindlin-2 loss decreases TMJ chondrocyte proliferation in condylar cartilages. Furthermore, Kindlin-2 loss promotes the release of cytochrome c as well as caspase 3 activation, and accelerates chondrocyte apoptosis in vitro and TMJ. Collectively, these findings reveal a crucial role of Kindlin-2 in condylar chondrocytes to maintain TMJ homeostasis.
Aggrecans/metabolism* ; Animals ; Cartilage, Articular/metabolism* ; Chondrocytes/pathology* ; Cytoskeletal Proteins/metabolism* ; Mice ; Muscle Proteins/metabolism* ; Osteoarthritis/pathology* ; Temporomandibular Joint/pathology*