Objective:To analyze the clinical characteristics, therapeutic responses, and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome (L-HES) .Methods:We retrospectively reviewed clinical data from 16 consecutive patients diagnosed with L-HES at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, between July 2019 and October 2024. A control group of 65 patients with idiopathic hypereosinophilic syndrome (iHES), diagnosed during the same period, was used for comparison. Clinical and laboratory characteristics, therapeutic responses, and survival outcomes were compared between the two groups.Results:The most frequently involved organs at presentation in patients with L-HES were the skin (75.0%), gastrointestinal tract (25.0%), respiratory tract (18.8%), lymph nodes (18.8%), heart (12.5%), and spleen (6.3%). Compared with iHES patients, patients with L-HES had a significantly higher incidence of skin involvement ( P=0.016), with no statistically significant differences observed in the involvement of other organs. No statistically significant differences were found in complete blood count parameters between the two groups. Multiparameter flow cytometry revealed that the median percentage of CD3 -CD4 + T cells in the peripheral blood of patients with L-HES was 4.08% ( IQR: 1.64%-32.78%), with a median absolute count of 0.10 (0.05-0.55) ×10 9/L. Serum immunoglobulin E (IgE) levels were significantly higher in the L-HES group than in the iHES group ( P<0.001). Clonal rearrangement of T-cell receptor genes was detected in 75.0% of patients with L-HES. After diagnosis, 14 patients with L-HES received glucocorticoids as first-line therapy, yielding an overall response rate of 92.9%. During glucocorticoid tapering, 11 patients experienced recurrent eosinophilia or worsening of clinical symptoms. Three patients received interferon-alpha as a second-line therapy, with two achieving complete remission. After a median follow-up of 16 months ( IQR: 8-28 months), one patient died of cardiac insufficiency 8 months after diagnosis, and no cases of lymphoma transformation were observed. The 2-year overall survival rate was (91.7±8.0) %, which did not significantly differ from that of the iHES group (96.2±2.6) % ( P=0.746) . Conclusions:Patients with L-HES generally have a favorable prognosis and are often characterized by skin involvement and significantly elevated serum IgE levels at diagnosis. They typically respond well to glucocorticoid therapy, although relapse is common during dose tapering. Interferon-alpha may serve as an effective second-line therapeutic option.

Objective:To analyze the differences between subjective refraction and autorefraction in myopic children and adolescents under different ciliary muscle functional states.Methods:A cohort study was conducted.A total of 98 myopic children and adolescents (196 eyes) aged 7-15 years who visited the Shanghai Eye Disease Prevention and Treatment Center from November 2023 to February 2024 were included by random sampling.All participants underwent cycloplegia with 1.0% cyclopentolate and completed both subjective refraction and autorefraction before cycloplegia, after cycloplegia and after recovery from cycloplegia.The spherical equivalent (SE) differences and differences in SE(ΔSE) between different conditions were compared.Proportion of ΔSE, differences in spherical power (ΔS), and differences in cylindrical power (ΔC) of objective and subjective refraction between different conditions within the clinically acceptable error range (-0.25 to 0.25 D) was calculated and compared.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Shanghai Eye Diseases Prevention & Treatment Center (No.2021SQ021).Written informed consent was obtained from guardian of each subject before any medical examination.Results:The SE values obtained from autorefraction before cycloplegia, after cycloplegia, and after recovery from cycloplegia were -2.44(-3.47, -1.63), -2.13(-3.25, -1.50), and -2.38(-3.50, -1.66)D, respectively, with a statistically significant overall difference ( χ2=148.36, P<0.001) and statistically significant differences in pairwise comparisons at different time points (all P<0.001); for subjective refraction, the SE values were -2.25(-3.50, -1.50), -2.19(-3.47, -1.45), and -2.28(-3.50, -1.50)D, respectively, with a statistically significant overall difference ( χ2=43.48, P<0.001) and statistically significant differences in pairwise comparisons at different time points (all P<0.001).Subjective refraction ΔSE between before and after cycloplegia, after cycloplegia and after recovery from cycloplegia were significantly smaller than those of autorefraction ( t=2.84, 1.82; both P<0.001).There was no significant difference in ΔSE between subjective refraction and autorefraction between before cycloplegia and after recovery from cycloplegia ( t=-0.43, P=0.070).The proportions of subjective refraction ΔSE within the acceptable error range between before and after cycloplegia, before cycloplegia and after recovery from cycloplegia, and after cycloplegia and after recovery from cycloplegia were significantly higher than those of autorefraction ( χ2=28.32, 11.82, 25.55; all P<0.001).The proportion of subjective refraction ΔS and ΔC both within the acceptable error range between before cycloplegia and after recovery from cycloplegia was 81.63%(160/196) and 79.59%(156/196) between after cycloplegia and after recovery from cycloplegia. Conclusions:Subjective refraction is less affected by different ciliary muscle functional states.The differences in subjective refraction results under different ciliary muscle functional states are mostly within the acceptable error range.The subjective refraction results before or after cycloplegia can be used to better predict the subjective refraction results after recovery from cycloplegia.

Objective:To analyze the differences between subjective refraction and autorefraction in myopic children and adolescents under different ciliary muscle functional states.Methods:A cohort study was conducted.A total of 98 myopic children and adolescents (196 eyes) aged 7-15 years who visited the Shanghai Eye Disease Prevention and Treatment Center from November 2023 to February 2024 were included by random sampling.All participants underwent cycloplegia with 1.0% cyclopentolate and completed both subjective refraction and autorefraction before cycloplegia, after cycloplegia and after recovery from cycloplegia.The spherical equivalent (SE) differences and differences in SE(ΔSE) between different conditions were compared.Proportion of ΔSE, differences in spherical power (ΔS), and differences in cylindrical power (ΔC) of objective and subjective refraction between different conditions within the clinically acceptable error range (-0.25 to 0.25 D) was calculated and compared.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Shanghai Eye Diseases Prevention & Treatment Center (No.2021SQ021).Written informed consent was obtained from guardian of each subject before any medical examination.Results:The SE values obtained from autorefraction before cycloplegia, after cycloplegia, and after recovery from cycloplegia were -2.44(-3.47, -1.63), -2.13(-3.25, -1.50), and -2.38(-3.50, -1.66)D, respectively, with a statistically significant overall difference ( χ2=148.36, P<0.001) and statistically significant differences in pairwise comparisons at different time points (all P<0.001); for subjective refraction, the SE values were -2.25(-3.50, -1.50), -2.19(-3.47, -1.45), and -2.28(-3.50, -1.50)D, respectively, with a statistically significant overall difference ( χ2=43.48, P<0.001) and statistically significant differences in pairwise comparisons at different time points (all P<0.001).Subjective refraction ΔSE between before and after cycloplegia, after cycloplegia and after recovery from cycloplegia were significantly smaller than those of autorefraction ( t=2.84, 1.82; both P<0.001).There was no significant difference in ΔSE between subjective refraction and autorefraction between before cycloplegia and after recovery from cycloplegia ( t=-0.43, P=0.070).The proportions of subjective refraction ΔSE within the acceptable error range between before and after cycloplegia, before cycloplegia and after recovery from cycloplegia, and after cycloplegia and after recovery from cycloplegia were significantly higher than those of autorefraction ( χ2=28.32, 11.82, 25.55; all P<0.001).The proportion of subjective refraction ΔS and ΔC both within the acceptable error range between before cycloplegia and after recovery from cycloplegia was 81.63%(160/196) and 79.59%(156/196) between after cycloplegia and after recovery from cycloplegia. Conclusions:Subjective refraction is less affected by different ciliary muscle functional states.The differences in subjective refraction results under different ciliary muscle functional states are mostly within the acceptable error range.The subjective refraction results before or after cycloplegia can be used to better predict the subjective refraction results after recovery from cycloplegia.

Objective:To analyze the clinical characteristics, therapeutic responses, and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome (L-HES) .Methods:We retrospectively reviewed clinical data from 16 consecutive patients diagnosed with L-HES at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, between July 2019 and October 2024. A control group of 65 patients with idiopathic hypereosinophilic syndrome (iHES), diagnosed during the same period, was used for comparison. Clinical and laboratory characteristics, therapeutic responses, and survival outcomes were compared between the two groups.Results:The most frequently involved organs at presentation in patients with L-HES were the skin (75.0%), gastrointestinal tract (25.0%), respiratory tract (18.8%), lymph nodes (18.8%), heart (12.5%), and spleen (6.3%). Compared with iHES patients, patients with L-HES had a significantly higher incidence of skin involvement ( P=0.016), with no statistically significant differences observed in the involvement of other organs. No statistically significant differences were found in complete blood count parameters between the two groups. Multiparameter flow cytometry revealed that the median percentage of CD3 -CD4 + T cells in the peripheral blood of patients with L-HES was 4.08% ( IQR: 1.64%-32.78%), with a median absolute count of 0.10 (0.05-0.55) ×10 9/L. Serum immunoglobulin E (IgE) levels were significantly higher in the L-HES group than in the iHES group ( P<0.001). Clonal rearrangement of T-cell receptor genes was detected in 75.0% of patients with L-HES. After diagnosis, 14 patients with L-HES received glucocorticoids as first-line therapy, yielding an overall response rate of 92.9%. During glucocorticoid tapering, 11 patients experienced recurrent eosinophilia or worsening of clinical symptoms. Three patients received interferon-alpha as a second-line therapy, with two achieving complete remission. After a median follow-up of 16 months ( IQR: 8-28 months), one patient died of cardiac insufficiency 8 months after diagnosis, and no cases of lymphoma transformation were observed. The 2-year overall survival rate was (91.7±8.0) %, which did not significantly differ from that of the iHES group (96.2±2.6) % ( P=0.746) . Conclusions:Patients with L-HES generally have a favorable prognosis and are often characterized by skin involvement and significantly elevated serum IgE levels at diagnosis. They typically respond well to glucocorticoid therapy, although relapse is common during dose tapering. Interferon-alpha may serve as an effective second-line therapeutic option.

Objective: Analysis of the molecular characteristics of eosinophilia. Methods: Targeting sequence to 24 patients with chronic eosinophilic leukemia (CEL) with rearrangement of PDGFRA, PDGFRB, or FGFR1 and 62 patients with hyper-eosinophilic syndrome (HES). Mutation annotation and analysis of amino acid mutation using authoritative databases to speculate on possible pathogenic mutation. Results: Thirty-seven kinds of clonal variant were detected from 17 patients with CEL, no recurrent mutation site and hot spot region were found. No pathogenic mutation was detected in 19 patients with PDGFRA rearrangement, but pathogenic mutations of ASXL1, RUNX1 and NRAS were detected from 2 patients with FGFR1 rearrangement who progressed to acute myeloid leukemia and 1 patient with PDGFRB rearrangement who progressed to T lymphoblastic lymphoma, respectively. One hundred and two kinds of clonal abnormalities were detected in 49 patients with HES. The main hot spot mutation regions included: CEBPA Exon1, TET2 Exon3, ASXL1 Exon12, IDH1 Y208C, and FGFR3 L164V. CRRLF2 P224L and PDGFRB R370C point mutations were detected separately in 2 patients with HES who treated with imatinib monotherapy and achieved hematologic remission. Conclusion The pathogenesis of CEL with PDGFRA, PDGFRB or FGFR1 rearrangement is usually single, and the progression of the disease may involve other driver mutation. A variety of genes with hot mutation regions may be involved in the pathogenesis of HES, and some mutation sites are sensitive to tyrosine kinase inhibitors.

In this study histochemical method was used. It has been found that on the seventh day after epidural administration of capsaicin substance p like immuno- reactivity (SPAR) in laminae Ⅰ-Ⅱ of dorsal horn of spinal cord decreased obv- iously as compared with that of vehicle treated animals, meanwhile, the activity of fluoride resistant acid phosphatase (FRAP) in both laminae Ⅱ of dorsal horn of spinal cord and the B-type cells of spinal ganglion disappeared. On the cont- rary the Leu-enkephalin immunoreactivity (ENK-IR) in laminae Ⅰ-Ⅲ of dorsal horn of spinal cord apparently increased as compared with that in vehicle group. the evidence from functional measurement showed that capsaicin can raise pain threshold, however, vehicle didnot have obvious influence on pain threshold. The foregoing results suggest that epidural administration of capsaicin in adult rat can effect the level of neurontransmitters, the activity of enzymes and block the con- duction of the primary afferent C fibers; the capsaicin-sensitive C fibers may have relationship with the ENK-containing interneurons in dorsal horn of spinal cord.