[Clinical and molecular characteristics and prognosis of classical hairy cell leukemia and hairy cell leukemia variant].

Objective: To evaluate the clinical characteristics, treatment response, and outcomes in patients with classical hairy cell leukemia (cHCL) and HCL variant (HCL-V). Methods: This is a retrospective case series study. Between January 2011 and December 2021, clinical data of 30 patients newly with diagnosed HCL at Peking Union Medical College Hospital were analyzed. The main outcome measures include clinical characteristics, treatment efficacy and survival. The Kaplan-Meier method was used for survival analysis. Results: Twenty-one cases of cHCL and 9 cases of HCL-v were included. The median age at diagnosis was 55.5 (range, 30-86) years, with the ratio of male to female 2.75∶1. The main clinical manifestations included fatigue in 11 cases (36.7%), abdominal distension in 7 cases (23.3%), and infection in 4 cases, while 8 cases were asymptomatic. Splenomegaly was reported in 24 cases (80.0%), including 7 (23.3%) with megalosplenia. The white blood cell count, lymphocyte count, and the proportion of peripheral hairy cells in HCL-v group were significantly higher than those in cHCL group, whereas the development of anemia, thrombocytopenia, and monocytopenia in cHCL group was more remarkable than that in HCL-v group (all P<0.05). The BRAF-V600E gene mutation was detected only in cHCL patients (11/14 vs. 0/9, P<0.001). In terms of immunophenotype, the expression of CD25, CD103, CD123 and CD200 in cHCL group (20/20, 20/20, 4/7, 7/17) were all stronger than those in HCL-v group (3/9, 7/9, 0/4, 2/8). Twenty-two patients were treated, of which 13 cases (12 cases of cHCL and 1 case of HCL-v) with cladribine, and 9 cases (4 cHCL and 5 HCL-v) with interferon. Complete remission rate and overall response rate were comparable between cladribine and interferon treatment groups (both P<0.05). The median follow-up time was 31 (range, 1-125) months, and the median overall survival (OS) of the entire group was 125 months. The 5-year OS rate in HCL-v patients represented a trend of inferior (50.0% vs. 95.0%, P=0.207). Conclusions: The clinical features of HCL are unspecific, which includes fatigue, splenomegaly and recurrent infection. The clinical features, immunophenotype, treatment response and prognosis of HCL-v are different from those of cHCL. BRAF-V600E gene mutation is suggested as a key marker for differential diagnosis. Cladribine is recommended as front-line regimen of cHCL patients with satisfactory efficacy and prognosis. Conversely, response and clinical outcome in HCL-v patients still need to be improved.

[The 502nd case: thrombocytopenia, splenomegaly, disseminated intravascular coagulation, and spontaneous rupture of the spleen].

A young man with a history of thrombocytopenia for seven years presented with splenomegaly and fever and rapidly evolved to disseminated intravascular coagulation (DIC) and hemorrhagic shock. Spontaneous rupture of the spleen was diagnosed. The critical patient underwent an emergency splenectomy. Pathological examination revealed splenic peliosis, an extremely rare disease with unknown etiology and pathogenesis. Despite the high mortality rate due to spontaneous splenic rupture with DIC, the patient was successfully treated and the details of the case are presented in this report.

[Expression of CYP2E1 in different brain regions during the formation of toxic cerebral edema induced by 1, 2-DCE in mice and its effect on cerebral edema formation].

Objective: To study the expression changes of cytochrome P-450 2E1 (CYP2E1) in different brain regions during 1, 2-dichloroethane (1, 2-DCE) exposure, and to explore the effect of CYP2E1 on the influence and effect of brain edema caused by 1, 2-DCE poisoning. Methods: In December 2018, forty female SPF Kunming mice were randomly divided into 4 groups: control group, 1 d, 2 d, and 3 d exposure groups, with 10 mice in each group. Placed in a 100 L static poisoning cabinet (5 animals/cabinet) , inhaled and exposed to the poison at 1.2 mg/L 1, 2-DCE for 3.5 h per day for 1 d, 2 d and 3 d. Except for exposure to 1, 2-DCE, other treatment methods were the same as those in the exposure groups. They were put to death the next day after the end of the poisoning, and the brain tissue was quickly removed and divided into sections. HE staining method was used for different brain regions. Western blotting method was used to detect the protein content of CYP2E1, occludin and claudin5 in different brain regions, real-time fluorescent quantitative PCR method was used to detect the mRNA expression levels of CYP2E1, occludin and claudin5. Histopathological observations were performed, and the kit method was used to detect malondialdehyde (MDA) , reduced glutathione (GSH) content and catalase (CAT) activity in different brain regions. The experimental data were analyzed by SPSS 20.0. One-way analysis of variance (one-way ANOVA) was used for the comparison of multiple groups, and the SNK (q test) method was used for the pairwise comparison between groups.P<0.05 was considered statistically significant. Results: Compared with the control group, histopathological observations showed obvious brain edema in the 2 d and 3 d exposure groups; Compared with the control group, the MDA content, CYP2E1 protein and mRNA expression levels in the cerebellar tissues of the mice in the 3 d exposure group were significantly increased, and the differences were statistically significant (P<0.05) ; Compared with the control group, the GSH content, CAT activity, occludin and claudin5 protein expression levels in the cerebellar tissues of the mice in each exposure group were significantly reduced, and the differences were statistically significant (P<0.05) . There was no significant difference in the above indicators of frontal cortex in each group in mice (P>0.05) . Conclusion: 1, 2-DCE can induce the expression of CYP2E1 in cerebellar tissues of mice, and cause oxidative damage and brain edema, but has no effect on the expression of CYP2E1 in frontal cortex of mice.

[Effect of anesthesia on cardiopulmonary complications in elderly patients with hip fracture].

Objective: To compare the effects of different types of anesthesia on postoperative cardiopulmonary complications in patients aged 80 years and above who received unilateral hip fracture surgery. Methods: The perioperative data of patients aged 80 and above who underwent unilateral hip fracture surgery in Peking Union Medical College Hospital from July 2009 to June 2014 were collected and grouped based on the types of anesthesia including general anesthesia, neuraxial anesthesia, and peripheral nerve block. Variables were analyzed including preoperative general conditions (age, sex, type of fracture, ASA physical status classification, preoperative cardiopulmonary complications), intraoperative conditions (type of surgical procedure, operative time, blood loss, total intraoperative fluid infusion), and postoperative conditions (length of hospital stay, postoperative hospital stay, ICU stay, postoperative newly developed cardiopulmonary complications, postoperative in-hospital death, hospitalization costs). The incidences of postoperative cardiopulmonary complications of each group were analyzed and compared. Results: A total of 219 cases were finally analyzed including general anesthesia (n=46), neuraxial anesthesia (n=90) and nerve block group (n=83). Fewer cases with preoperative pulmonary complications were noticed in general anesthesia group (15 cases, 32.6%) than in neuraxial anesthesia group (45 cases, 50%) and nerve block group (47 cases, 56.6%) (χ(2)=6.912, P=0.032). No statistical differences were found between groups regarding preoperative cardiovascular complications and other variables. There were no significant differences in postoperative cardiopulmonary complications. However, the cost of hospitalization was lower in neuraxial anesthesia group (45.6±21.4) thousand RMB and nerve block group (48.2±25.8) thousand RMB compared with general anesthesia group (56.3±21.6) thousand RMB, the difference was statistically significant(F=9.951, P=0.007). Conclusions: For elderly patients undergoing unilateral hip surgery, the type of anesthesia does not affect the incidence of postoperative cardiopulmonary complications. However, neuraxial anesthesia and nerve block may reduce the cost of hospitalization.

[Efficacy and safety of transbronchial cryobiopsy in the etiologic diagnosis of diffuse lung disease].

Objective: To assess the efficacy and safety of transbronchial cryobiopsy (TBCB) for the etiologic evaluation of diffuse lung disease (DLD). Methods: Between December 2015 to April 2017, a total of 38 patients with DLD met the inclusion criteria for TBCB in the First Affiliated Hospital of Chongqing Medical University, and 35 of them consented to undergo the procedure under rigid or flexible bronchoscopy. On the tissues obtained from the 35 patients, histopathologic and microbiological evaluations were performed, and together with clinical and radiological manifestations, diagnoses were made and the efficacy of TBCB in the diagnosis of DLD was confirmed, and then therapies were planned accordingly. Complications of the biopsy procedures were recorded. Results: Of the 35 patients who were enrolled, 24 underwent TBCB under rigid bronchoscopy and 11 under flexible bronchoscopy. Another 3 patients refused the procedure due to disinclination to invasive examinations. One single procedure of TBCB took (51.8±19.2) min on average, the median number of tissues obtained was 6 (5, 8), and the median area of tissues was 15 (9, 20) mm(2).Definite diagnoses were reached in 33 patients, including idiopathic nonspecific interstitial pneumonia (n=8), connective tissue disease-interstitial lung disease (n=8), occupational lung disease (n=4), idiopathic pulmonary fibrosis (n=3), interstitial pneumonia with autoimmune features (n=3), tuberculosis (n=2), cryptogenic organization pneumonia (n=1), acute interstitial pneumonia (n=1), pulmonary infection (n=1), hypersensitivity pneumonia (n=1) and sarcoidosis (n=1). Diagnostic yield was 94.3% (33 out of 35 cases diagnosed). Pneumothorax occurred in 3 patients (1 patients with mild pneumothorax , 1 moderate and 1 severe), and were resolved with thoracic puncture or pleural drainage. Bleeding occurred in all 24 patients who received TBCB under rigid bronchoscopy (11 patients with mild bleeding, 12 moderate and 1 severe) and was controlled after coagulation measures. After one month of treatment according to the diagnoses acquired with cryobiopsy, the condition was cured in 1 patient (3.0%), alleviated in 17 (51.5%), stable in 11 (33.3%), and deteriorated in 4 (12.1%). Conclusion: TBCB yields reliable diagnoses with a good safety profile.

Initial conditions for dark soliton generation in normal-dispersion fiber lasers.

We report results of numerical simulations on the various initial conditions for dark soliton generation in an all-normal-dispersion fiber laser. All the dark solitons generated are odd dark solitons. Differently from the dark soliton generation in fibers, where an arbitrary dip could evolve into a dark soliton, it is found that the dark soliton can originate only from an initial dip with a certain parameter requirement. A bright pulse with either a hyperbolic secant square, Gaussian, or Lorentz profile can be developed into a dark soliton, provided that the parameters of the initial bright pulse are selected. Dark solitons can be generated in fiber lasers only if there is a phase jump, and this phase jump can be maintained and evolve to π during the pulse evolution.

Universal primers to amplify the complete mitochondrial 12S rRNA gene in marine fish species.

A pair of new universal 12S mitochondrial rRNA gene primers was designed through multiple alignment analysis of the mitochondrial tRNA(Phe) and the 5' region of 16S mitochondrial rRNA genes of different kinds of fishes. The primers were successfully used to amplify an expected product fragment of about 1.2 kb from various marine fish species, and the amplified DNA fragment was recognized to contain the complete 12S mitochondrial rRNA and tRNA(Val) genes, as well as a partial 16S mitochondrial rRNA gene of about 146 bp in length. The primers would facilitate the study of the species discrimination, population and evolution in marine fish species.

Universal primers for amplification of the complete mitochondrial control region in marine fish species.

Through multiple alignment analysis of mitochondrial tRNA-Thr and tRNA-Phe sequences from 161 fishes, new universal primers specially targeting the entire mitochondrial control region were designed. This new primer set successfully amplified the expected PCR products from various kinds of marine fish species, belonging to various families, and the amplified segments were confirmed to be the control region by sequencing. These primers provide a useful tool to study the control region diversity in economically important fish species, the possible mechanism of control region evolution, and the functions of the conserved motifs in the control region.

The preparation, cytocompatibility, and in vitro biodegradation study of pure beta-TCP on magnesium.

Biodegradable and bioactive beta-tricalcium phosphate (beta-TCP) coatings were prepared on magnesium (Mg) in order to improve its biocompatibility by a chemical method. The tensile bonding strength of beta-TCP coating and Mg substrate was measured by the standard adhesion test (ISO 13779-4). And the cytocompatibility of beta-TCP coated Mg was studied by using human osteoblast-like MG63 cells. It was found that the MG63 cells could grow well on the surface of beta-TCP coated Mg and the cell viability on beta-TCP coated Mg was above 80% during the cocultivation of MG63 cells and beta-TCP coated Mg for 10 days, indicating no cytotoxicity. It was concluded that the beta-TCP coated Mg had good cytocompatibility. The degradation of Mg substrate with beta-TCP coating in vitro was studied in detail by XRD, EDX, SEM, and ICP. The results showed that a bone-like apatite continually formed on the surface of the sample with the degradation of both Mg substrate and beta-TCP coating in Hank's solution (a simulated body fluid). The biodegradation mechanism was preliminarily analyzed in the paper.

An outbreak of paragonimiosis in Zhengzhou city, China.

An outbreak of paragonimiosis occurred in the city of Zhengzhou, Central China, in May 1995. Of seven exposed urban inhabitants, four were seropositive for Paragonimus and had clinical symptoms consistent with acute paragonimiosis. All of four cases treated were cured with praziquantel. The P. skrjabini adult worm was obtained from the rats experimentally infected with the crabs collected in the mountainous area where the urban inhabitants had got the infection. The outbreak was attributed to the intake of raw freshwater crabs while on tourist trips in a mountainous area.

Epidemiological, clinical and serological studies on trichinellosis in Henan Province, China.

The epidemiological, clinical and serological studies on human trichinellosis in Henan Province were carried out from January 1992 to December 1996. The results showed that 467 patients attending our department came from 12 administrative areas and cities of the Province and they acquired the infection mainly by eating undercooked dumplings, tasting the raw pork filling for dumplings, ingesting scalded pork or mutton. There was the high incidence season of trichinellosis during the winter. Most of the patients were workers, officers and merchants aged 20-49 years, and the incidence was higher in males than in females. The main clinical manifestations of trichinellosis were fever, general myalgia, muscle tiredness and eosinophilia. Most of the patients had not any gastrointestinal symptoms and rash. Eyelid edema was only seen in the early courses of the disease. Eosinophil count increased with time, reached to the peak 3 weeks and decreased markedly 6 weeks after the onset of the disease. Eosinophil levels elevated obviously 1 week after therapy, began to drop rapidly 2 weeks after therapy, and decreased to a near normal levels 4 weeks after therapy. Anti-Trichinella antibodies were detected by IFAT using the frozen sections of the purified T. spiralis larvae as antigen. The specific antibodies were observed in only 70.2% of patients one week after onset of disease, and increased to 91, 94.3, and 100%; 2, 3, and 4 weeks, respectively after the onset. The antibody positive rate was evidently increased to 100% 1 week after therapy with abendazole, then decreased to 25% 4 months after therapy.

[Detection of serum antibodies in rats infected with Angiostrongylus cantonensis by indirect fluorescent antibody test].

Frozen section specimens with a thickness of 4 microns were made from adult worms of Angiostrongylus cantonensis in order to detect the serum antibodies in rats infected with Angiostrongylus cantonensis by indirect fluorescent antibody test (IFAT). The results showed that the positive rates of the serum samples 2 and 4 weeks after infection were 92.0% (46/50) and 100%(19/19), respectively. The results of serum samples in healthy rats and normal saline (as control) were negative. This technique proved to have a highly detectable rate and the positive reaction might occur in early infection. It might be used for the early supplementary diagnosis of angiostrongyliasis.

[Experimental study on detection of serum antibodies by immunoenzyme staining using adult Angiostrongylus cantonensis frozen section].

This paper reported on the results of detection of serum antibodies in rats with Angiostrongylus cantonensis infection by immunoenzyme staining of adult frozen section. The positive rates of serum antibodies in rats 2 and 4 weeks after infection were 80% and 93.3%, respectively. The result of healthy control rats was negative.

Epidemiological and clinical studies on an outbreak of trichinosis in central China.

An outbreak of trichinosis occurred in the city of Zhengzhou, central China, between December 1995 and February 1996, affecting 85 of the administrative units into which the city is split. Of 297 subjects from eight of the affected units, 54% were seropositive for Trichinella and 41% had symptoms consistent with acute trichinosis. Of the 490 subjects who had eaten at one particular dumpling restaurant 1-5 weeks before the outbreak and who were traced, 291 (59%) were seropositive and 212 (43%) had been or were ill. MOst of the infections were in manual workers, cadres and merchants aged 20-49 years. Most of those who had been infected failed to develop gastro-intestinal symptoms or a cutaneous rash. Eyelid oedema was only seen in the early stages of the infection, the main clinical manifestations being fever of long duration of tiredness. Surprisingly, six cases had no marked symptoms after repeated infection. Eosinophilia (eosinophils > 7% of leucocytes) was noted in 71 (55%) of the 130 cases in which blood cells were counted. When 212 sera were tested for antibodies to Trichinella, seropositivities were found to increase from 89.1% (IFAT) of 87.7% (microprecipitation test) at presentation to 100% (both tests) 1 week after treatment with albendazole. All those treated were cured. The outbreak was one of the most extensive, single-source outbreaks ever recorded in China, probably with > 600 infections and > 300 clinical cases. The entire episode was attributed to the ingestion of undercooked pork dumplings at one restaurant.