Follicular lymphoma (FL) is a kind of indolent Non-Hodgkin lymphoma (NHL),which stems from follicle germinal center.A lot of research reported the latest development about treatment of FL in the 56th American Society of Hematology (ASH) annual meeting.Effect of radiotherapy for patients at low stage was determined,while the outcomes of clinical trials in which monoclonal antibody (GA101) in combination with chemotherapy for the advanced stage FL were exciting.In rituximab era,relapsed/refractory FL patients who receive stem cell transplantation could acquire preferable remissions.Several novel drugs (inhibitor of BTK or PI3K) for relapsed/refractory FL played a role of guidepost with the progression of corresponding trials.

OBJECTIVE: To analyze the correlation between the mutational status of immunoglobulin heavy chain variable (IGHV) gene with the prognosis of patients with Waldenström macroglobulinemia (WM). METHODS: Immunoglobulin heavy chain gene (IGH) clonotypic sequence analysis was carried out to assess the mutational status of IGHV in the blood and/or bone marrow samples from 44 WM patients. The usage characteristics of IGHV-IGHD-IGHJ gene was explored. RESULTS: The most common IGHV subgroup was IGHV3, which was similar to the data from the Institute of Hematology of Chinese Academy of Medical Science. IGHV3-23 (20.45% vs. 15.44%) and IGHV3-74 (11.36% vs. 7.35%) were the main fragments used, which was followed by IGHV4 gene family (15.91% vs. 24.26%). However, no significant correlation was found between the IGHV4 usage and the prognosis of the patients. Should 98% be taken as the cut-off value for the IGHV mutation status, only 5 patients had no IGHV variant, and there was no correlation with the prognosis. Based on the X-tile analysis, 92.6% was re-selected as the cut-off value for the IGHV variant status in such patients. LDH was increased in 26 patients (59.1%) without IGHV variant (P < 0.05), whilst progression-free survival (P < 0.05) and overall survival (P < 0.05) were significantly shorter compared with those with IGHV variants. CONCLUSION The usage characteristics of IGHV-IGHD-IGHJ in our patients was similar to reported by the Institute of Hematology of Chinese Academy of Medical Science, albeit that no correlation was found between the IGHV4 usage and the prognosis of the patients. Furthermore, 98% may not be appropriate for distinguishing the IGHV variant status in WM patients.
Humans ; Immunoglobulin Heavy Chains/genetics* ; Multigene Family ; Mutation ; Waldenstrom Macroglobulinemia/genetics*

Objective To investigate the electro-clinical features of epilepsy with eye closure sensitivity (ECS).Methods The electroencephalograph database was searched using ECS during half a year period from January to June 2017 in Xijing Hospital.The duration of the follow-up was one year.Results Fifty-one patients diagnosed as epilepsy with ECS were investigated.Patients were classified into four epilepsy syndromes:33 with eyelid myoclonia with absences (EMA);13 with juvenile myoclonia epilepsy (JME);two with epilepsy with generalized tonic-clonic seizure on awakening and three with idiopathic occipital lobe epilepsy (IOE).The duration of the epileptiform discharges (EDs) triggered by eye closure (ECL) lasted more than five seconds in four patients with EMA and one patient with IOE.The EDs triggered by ECL were more frequent (85.2± 11.4 vs 37.5± 12.6,t=12.399,P=0.000) and lasting longer ((4.3± 1.9) s vs (2.3±0.8) s,t=3.585,P=0.001) in EMA than in JME.Conclusions ECS is common in EMA.The frequency and duration of the EDs triggered by ECL are helpful for identifying EMA and JME.

Objective:To investigate the effect of obesity on the efficacy of allogeneic hematopoietic stem cell transplantation.Methods:The clinical data of 81 patients who underwent allogeneic hematopoietic stem cell transplantation from August 2017 to September 2020 in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed. According to the body mass index (BMI), the patients were divided into the obese group (BMI≥28 kg/m 2, 11 cases) and the non-obese group (BMI<28 kg/m 2, 70 cases). The clinicopathological characteristics, hematopoietic stem cell implantation, post-transplantation complications, survival and recurrence were compared between the two groups. Univariate and multivariate survival analyses were performed by using Cox proportional hazards regression model. Results:The median follow-up time of 81 patients was 280 d (8-1 218 d). The 1-year overall survival (OS) rate was 77.9%, and the 1-year progression-free survival (PFS) rate was 73.8%. The 1-year OS rates of the non-obese group and the obese group were 82.6% and 46.2% ( χ2 = 15.54, P<0.01), and the 1-year PFS rates were 82.1% and 36.4% ( χ2 = 15.56, P<0.01). The non-recurrence mortality (NRM) rates of the non-obese group and the obese group were 7.1% and 32.7% ( χ2 = 6.463, P = 0.01), and the cumulative recurrence rate was 11.5% and 42.9% ( χ2 = 8.146, P = 0.004). Between the non-obese group and the obese group, the median engraft time of neutrophils and platelets, acute graft-versus-host disease, chronic graft-versus-host disease, hemorrhagic cystitis, cytomegalovirus infection and Epstein-Barr virus infection had no statistical difference ( P > 0.05). The result of multivariate analysis showed that obesity was an independent adverse influencing factor for OS of patients with allogeneic hematopoietic stem cell transplantation ( HR = 3.814, 95% CI 1.343-10.827, P = 0.012). Conclusion:Obesity is an important unfavorable factor that affects patient's survival after allogeneic hematopoietic stem cell transplantation, and the improvement of the efficacy and survival of these patients is worthy of further study.

Objective:To investigate the clinical effect of the "Tongluo" technique on gastrocnemius muscle hardness and muscle tone in children with spastic cerebral palsy (SCP) using shear wave elastography (SWE).Methods:Forty children with SCP who were treated at the First Affiliated Hospital of Tianjin University of Traditional Chinese Medicine from January 2020 to June 2021 were selected as the observation group and received the "Tongluo" technique intervention. At the same time, 20 normal children were selected as the control group, without any intervention. Compare the various indicators of the two groups at baseline and after 1.5 and 3 months of treatment, including Young’s modulus value of the medial gastrocnemius muscle at rest, the modified Ashworth scale (MAS) score, and the clinical spasm index (CSI) score. The changes in muscle tone and spasticity of SCP children before and after treatment were compared, and the differences in the Young’s modulus between MAS and CSI levels in SCP children as well as the correlation between MAS, CSI, and Young’s modulus were analyzed.Results:The difference in Young’s modulus values of the control group at baseline, after 1.5 and 3 months of treatment was not statistically significant (all P > 0.05). At baseline, after 1.5 and 3 months of treatment, Young’s modulus value, MAS score, and total CSI score gradually decreased in the observation group, and the differences obtained from pairwise comparisons were statistically significant (all P < 0.01). At the same intervention stage, Young’s modulus value of the observation group was higher than that of the control group, with a statistically significant difference ( P < 0.01). The MAS score results showed that Young’s modulus of the observation group increased with the increase in muscle tone, and there was a positive correlation between Young’s modulus and muscle tone ( P < 0.01), and the difference in Young’s modulus corresponding to different muscle tones was statistically significant ( P < 0.05). The CSI score results showed that the Young’s modulus of the observation group increased with the increase in spasticity degree, and there was a positive correlation between the Young’s modulus and muscle tone ( P < 0.01), and the difference in Young’s modulus corresponding to different spasticity degrees was statistically significant ( P < 0.05). Conclusions:The muscle hardness of children with SCP is higher than that of normal children. The "Tongluo" technique can effectively improve muscle hardness, reduce muscle tone, and effectively improve muscle spasticity in children with SCP. The principle may be related to adjusting the internal structure of muscles to reduce muscle hardness. The results of this study can guide clinical rehabilitation and evaluation.

The tumor microenvironment (TME) is comprised of tumor cells, immune cells and stromal cells, moreover the intricate interactions among these cellular components play a vital role in tumor initiation and progression. Within the TME, immune cells and stromal cells engage in cytotoxic or inflammatory responses to counteract tumor cells, but they employ a range of immune regulatory mechanisms to facilitate tumor evasion. Previous treatments for lymphoma mainly targeted malignant cells themselves. However, with the clinical application of immune checkpoint inhibitors, bispecific antibodies and chimeric antigen receptor T-cell therapy, the elimination of TME-mediated tumor protection has gained increasing attention. By harnessing the high-throughput and multi-dimensional analytical capabilities of mass cytometry (CyTOF) and imaging mass cytometry (IMC), it has become feasible to systematically analyze the composition of the lymphoma TME using large-scale samples.

Humans ; Hematopoietic Stem Cell Transplantation ; Transplantation, Autologous ; Lymphoma, Non-Hodgkin/drug therapy* ; Aminopyridines/therapeutic use* ; Antineoplastic Combined Chemotherapy Protocols

Mutations or inactivation of parkin, an E3 ubiquitin ligase, are associated with familial form or sporadic Parkinson's disease (PD), respectively, which manifested with the selective vulnerability of neuronal cells in substantia nigra (SN) and striatum (STR) regions. However, the underlying molecular mechanism linking parkin with the etiology of PD remains elusive. Here we report that p62, a critical regulator for protein quality control, inclusion body formation, selective autophagy and diverse signaling pathways, is a new substrate of parkin. P62 levels were increased in the SN and STR regions, but not in other brain regions in parkin knockout mice. Parkin directly interacts with and ubiquitinates p62 at the K13 to promote proteasomal degradation of p62 even in the absence of ATG5. Pathogenic mutations, knockdown of parkin or mutation of p62 at K13 prevented the degradation of p62. We further showed that parkin deficiency mice have pronounced loss of tyrosine hydroxylase positive neurons and have worse performance in motor test when treated with 6-hydroxydopamine hydrochloride in aged mice. These results suggest that, in addition to their critical role in regulating autophagy, p62 are subjected to parkin mediated proteasomal degradation and implicate that the dysregulation of parkin/p62 axis may involve in the selective vulnerability of neuronal cells during the onset of PD pathogenesis.
Adaptor Proteins, Signal Transducing ; chemistry ; metabolism ; Animals ; HEK293 Cells ; Heat-Shock Proteins ; chemistry ; metabolism ; Humans ; Lysine ; metabolism ; Mice ; Neurons ; metabolism ; pathology ; Oxidopamine ; pharmacology ; Parkinson Disease ; metabolism ; pathology ; Proteasome Endopeptidase Complex ; metabolism ; Protein Stability ; Proteolysis ; drug effects ; Sequestosome-1 Protein ; Ubiquitin-Protein Ligases ; metabolism ; Ubiquitination ; drug effects