Objective To investigate the expressions of tenascin(TN)and microvessel density(MVD) in esophageal squamors cell carcinoma.Methods The experessions of TN and MVD were observed by immunohistochemiccal methods in 91 cases of esophageal carcinoma and 47 cases of ono-esophageal carcinoma.Results ①The expression of tenascin in esophageal cancer was much high than that in t he normal tissue(t=12.331,P＜0.01).②Over expression of TN was related to th length(F=12.373,P＜0.01),the invasion(F=11.039,P＜0.01),the lymphatic metastasis(F=6.882,P=0.01)and the pathologic trade(F=5.060,P=0.003)of cancer.③The expression fo MVD in the esophageal carcinoma tissues was stonger than that in the nonesophageal carcinoma tissues(t=6.023,P＜0.01).④Over expression of NVD wsa related to the length(F=9.033,P＜0.01),the lymphatic metastasis(F=12.429,P＜0.01)and the pathologic grade(F=5.717,P＜0.01)of cancer.⑤The positive expressions of TN and MVD were associated(P=0.001).Conclusion TN;s voerexpressions may be a biological marker in esophageal squamous cell carcinoma..It was associated with the expression of MVD.The levels of TN and NVD were useful molecular markers for evaluating malignancy degree and lymph node metastasis of esopageal carcinoma.

BACKGROUND: Esophageal replacement or reconstruction should be performed after esophageal resection. There are still no suitable substitutes for esophagus if the conventional esophageal substitutes cannot be used.OBJECTIVE : To investigate the feasibility of applying a pulmonary tissue with vascular pedicle to repair the intrathoracic esophageal defect.DESIGN: A prospective animal investigation.SETTING: Department of Thoracic Surgery, Second Hospital Affiliated to China Medical University.MATERIALS: This trial was carried out in the laboratory of Department of Thoracic Surgery, Second Hospital Affiliated to China Medical University during January 2003 to June 2004. Fourteen adult mongrel dogs of either gender, with body mass of 12 to 18 kg, were provided by Animal Room, Second Hospital Affiliated to China Medical University (License No.SYXK (Liao) 2003-0019).METHODS: Of 14 anesthetized dogs, the middle lobe of right lung was dissected and its right middle lobar bronchus was ligated without damaging pulmonary and bronchial vessels in order to make pulmonary flap. A part of full-layer intrathoracic esophageal wall was resected, which was 4 cm long and 1/2 to 2/3 circled esophageal wall. The defect was patched by pulmonary tissue with vascular pedicle which was inosculated with esophageal cross section. On the 3rd day after operation, intravenous transfusion was performed to maintain nutrition. Qn the 7th day after operation, the dogs were given oral liquid soft food gradually 2 weeks after the operation. The access to the food and the survival of dogs were observed. Every 2 dogs were sacrificed respectively at the 2nd, 4th, 6th, 8th and 10th postoperative weeks. To observe the healing of esophageal defect, light microscope, transmission electron microscope, esophagography and endoscope were used in this study.MArN OUTCOME MEASURES: ①Survival situation and access to food of dogs after operation. ② The healing of esophageal defect of dogs.RESULTS: Three of fourteen dogs died within one week after operation. Eleven dogs survived. ① The survival and access to food of experimental dogs after operation: One dog was alive without problems for more than 170 weeks. The living dogs could be fed orally on the 7th day after operation. ② The healing at esophageal defect of experimental dogs:At the 2nd week after operation, the esophageal defect was covered with collagen layer and inflammatory exudation. A little epithelization was observered at free edge of the anastomosis, which was 1 to 2 layers of stratified squamous epithelium cells. At the 4th to 6th weeks after operation, the internal surface of the defect was covered with 3 to 5 layers of stratified epithelium cells. At the 8th to 10th weeks after operation, the luminal surface of the defect was covered with 6 to 8 layers of stratified epithelium cells. The pathological changs of pulmonary flap mainly included pulmonary alveoli atelectasis and pulmonary fibrosis, and some inflammatory cells without infective focus were observed. In the transmission electron microscope examination, newborn stratified squamous epithelium cells were. found on the surface of pulmonary tissue flap at esophageal defect.CONCLUSION: It is feasible to repair the partial irregular intrathoracic esophageal defect with the autologous pulmonary flap in dogs.

OBJECTIVETo observe the influence of flap-on or flap-off Epipolis laser in situ keratomileusis (epi-LASIK) on the release of transforming growth factor-β1 (TGF-β1) in tear fluid and corneal haze formation.

METHODSThirty patients (60 eyes) with myopia underwent epi-LASIK surgery with epithelial flap repositioning (flap-on) in the right eyes and epithelial flap removal (flap-off) in the left eyes. The level of TGF-β1 in tears was measured preoperatively and on days 1, 3, and 7 postoperatively. Corneal haze was graded at 1, 3 and 6 months after surgery.

RESULTSThe mean preoperative spherical equivalent refraction was -4.98∓2.28 D (-2.50 to -7.25 D) in flap-on group and -5.20∓4.02 D (-1.75 to -7.00 D) in flap-off group, showing no significant difference between the two groups (P=0.80). TGF-β1 levels in the tear fluid were similar in the two groups preoperatively (P=0.11) and at 1, 3, and 7 days postoperatively (P=0.55, 0.45, 0.19, respectively). TGF-β1 levels in tears gradually decreased after the first postoperative day in both groups, but were still higher than the preoperative value till the 7th postoperative day. Corneal haze scores in the two groups were similar at 1 month (P=0.98), 3 months (P=0.52), and 6 months (P=0.72) after the operation.

CONCLUSIONFlap-on and flap-off epi-LASIK surgeries do not differ significantly in postoperative TGF-β1 levels in the tear fluid or in the postoperative haze scores. TGF-β1 may play a role in corneal wound healing.

Adult ; Cornea ; surgery ; Epithelium, Corneal ; pathology ; surgery ; Female ; Humans ; Keratomileusis, Laser In Situ ; methods ; Male ; Postoperative Period ; Surgical Flaps ; Tears ; metabolism ; Transforming Growth Factor beta1 ; metabolism ; Young Adult

OBJECTIVETodelineate the clinical and genetic features of a patient with myeloproliferative neoplasm (MPN) in association with PDGFRA and EVI1 genes rearrangements.

METHODSClinical data of the patient was collected. Conventional cytogenetics, fluorescence in situ hybridization (FISH) and nested PCR were carried out for the patient.

RESULTSThe patient has featured recurrent rash, joint pain, and intermittent fever. Laboratory tests showed hyperleukocytosis and marked eosinophilia. Physical examination revealed splenomegaly. His karyotype was 46,XY,t(3;5)(q26;q15)[6]/46,XY[10]. FISH assay showed that both PDGFRA and EVI1 genes were rearranged. Molecular studies of the mRNA suggested that there was a in-frame fusion between exon 12 of the PDGFRA gene and exon 9 of the FIP1L1 gene. Imatinib was initiated at a dosage of 200 mg, and after 10 months, the signal of the FIP1L1-PDGFRA fusion gene was undetectable in bone marrow sample. However, the expression of EVI1 mRNA was stable, with no significant difference found between the patient and 10 healthy controls.

CONCLUSIONMPN in association with PDGFRA and EVI1 genes rearrangements have unique clinical and genetic features. Genetic testing is helpful for early diagnosis. Imatinib may be effective for the treatment.

Antineoplastic Agents ; therapeutic use ; Base Sequence ; Chromosome Banding ; Chromosomes, Human, Pair 3 ; genetics ; Chromosomes, Human, Pair 5 ; genetics ; DNA-Binding Proteins ; genetics ; Gene Rearrangement ; Humans ; Imatinib Mesylate ; therapeutic use ; In Situ Hybridization, Fluorescence ; Karyotyping ; MDS1 and EVI1 Complex Locus Protein ; Male ; Myeloproliferative Disorders ; drug therapy ; genetics ; Proto-Oncogenes ; genetics ; Receptor, Platelet-Derived Growth Factor alpha ; genetics ; Transcription Factors ; genetics ; Translocation, Genetic ; Treatment Outcome ; Young Adult

Objective: To analyze the incidence and microbiological features of blood stream infections (BSI) of patients with hematopoietic stem cell transplantation (HSCT) and to provide laboratory data for empirical use of antibiotic for the HSCT patients with BSI. Methods: The incidence of bloodstream infection, the positive rate of blood culture, bacterial spectrum and drug resistance were analyzed in 1 265 HSCT recipients during 2013 and 2015 were retrospectively studied. Results: Of 1265 patients undergoing HSCT, 1 422 cases of suspected BSI occurred in 784 patients (61.98%) , and 464 patients (59.2%) were in the stage of agranulocytosis (ANC<0.5×10⁹/L) . The detection rate of pathogens in 2013-2015 was about 20% and increase year after year. Of the 401 strains detected, 221 were Gram-negative (G^-) bacteria (55.1%) , 165 Gram-positive (G⁺) bacteria (41.2%) and 15 fungi (3.7%) . Escherichia coli (16.0%) , Staphylococcus epidermidis (15.5%) and Klebsiella pneumoniae (11.2%) were listed the top three. The proportion of multidrug resistant Acinetobacter Bauman and Stenotrophomonas maltophilia was 64.70% and 63.64% respectively, and methicillin resistant Staphylococcus aureus (MRSA) was more than half (57.14%) . The ratio of vancomycin resistant Enterococci (VRE) and carbapenem resistant Enterobacteriaceae (CRE) was 14.29% and 6.78% respectively. More than 40% Enterobacteriaceae bacteria were resistant to three or four generation cephalosporin antibiotics, and less were resistant to the carbapenems (6.4%) . However, many non-fermentating bacteria were highly resistant to these antibiotics and showed diversity among different strains, with a rate of 47.8% resistance to carbapenems. All the Staphylococcus were sensitive to vancomycin, teicoplanin and linezolid. Conclusions The incidence of BSI in patients with HSCT was high, and the pathogens were mainly G^- bacteria. In addition to Enterobacteriaceae, the proportion of non-fermentative bacteria was quite high. No Staphylococcus detected were resistant to vancomycin, teicoplanin and linezolid.

Objective: To compare the outcomes between haploidentical donor hematopoietic stem cell transplantation (haplo-HSCT) and matched-sibling donor transplantation (MSD-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) . Methods: The clinical data of 40 PNH patients received HSCT (haplo-HSCT=25, MSD-HSCT=15) from July 2007 to May 2018 were analyzed retrospectively to compare the outcomes between haplo-HSCT and MSD-HSCT groups. Results: There were no differences in terms of gender, age, patients of PNH-AA and median time from diagnosis to transplantation between the 2 groups (P>0.05) . The median values of absolute mononuclear cell counts and CD34⁺ cells infused were 10.74 (4.80-22.86) ×10⁸/kg and 12.19 (5.14-17.25) ×10⁸/kg (P=0.866) , 3.57 (0.68-7.80) ×10⁶/kg and 4.00 (3.02-8.42) ×10⁶/kg (P=0.151) respectively, in haplo-HSCT and MSD-HSCT groups. All patients attained complete engraftment, no patient occurred graft failure. The median durations for myeloid and platelet engraftment were 12 (range, 9-26) and 11 (range, 7-15) days (P=0.065) , 19 (range, 11-75) and 13 (range, 11-25) days (P=0.027) respectively, in haplo-HSCT and MSD-HSCT groups. During a median follow-up of 26 (4-65) months in haplo-HSCT and 36 (4-132) months in MSD-HSCT groups (P=0.294) , the incidences of grade Ⅰ-Ⅳ acute graft-versus-host disease (aGVHD) were 32.0% and 20.0% (P=0.343) , grade Ⅱ-Ⅳ aGVHD were 16.0%, 13.3% (P=0.759) , chronic GVHD were 30.7% and 24.6% (P=0.418) , moderate-severe chronic GVHD were 12.7% and 7.1% (P=0.522) respectively, in haplo-HSCT and MSD-HSCT groups. The incidences of infection were 32.0% (8/25) and 26.7% (4/15) (P=1.000) respectively, in haplo-HSCT and MSD-HSCT groups. No patients occurred early death and relapse. Three-year estimated overall survival (OS) were (86.5±7.3) % and (93.3 ±6.4) % (P=0.520) , GVHD-free and failure-free survival (GFFS) were (78.3±8.6) % and (92.9±6.9) % (P=0.250) respectively, in haplo-HSCT and MSD-HSCT groups. Conclusion The preliminary results indicated that haplo-HSCT was a feasible choice for PNH with favorable outcomes, haplo-HSCT and MSD-HSCT produced similar therapeutic efficacy.

Objective: To compare the outcomes of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) with paroxysmal nocturnal hemoglobinuria-aplastic anemia (PNH-AA) syndrome. Methods: The outcomes of 46 patients who received allo-HSCT (16 PNH patients, 30 PNH-AA patients) from July 10, 2007 to June 2, 2018 were analyzed retrospectively. The conditioning regimen was busulfan, cyclophosphoramide, and ATG in haploidentical donors and unrelated donors. Patients with matched sibling donors were treated with the fludarabine, cyclophosphamide, and ATG regimen. Results: There were no differences of baseline data between the 2 groups except gender distribution and the numbers of haploidentical donor transplantation. The median values of absolute nucleated cell counts were 10.58 (3.83-13.83) ×10⁸/kg in the PNH group and 10.81 (3.96-33.40) ×10⁸/kg in the PNH-AA group (P=0.668) . The median doses of CD34⁺ cells infused were 5.00 (3.14-8.42) ×10⁶/kg and 3.57 (1.97-6.17) ×10⁶/kg (P=0.002) , respectively. All patients obtained complete engraftment. The median time for myeloid engraftment were 11 (7-14) days in the PNH group and 12 (10-26) days in the PNH-AA group (P=0.003) . The median time for platelet engraftment were 13 (11-16) days and 18 (12-75) days (P=0.002) , respectively, after a median follow-up of 36 (4-132) months in the PNH group and 26 (4-75) months in the PNH-AA group (P=0.428) . There were no differences of incidence rates of acute graft-versus-host disease (aGVHD) , chronic GVHD and infection between PNH and PNH-AA groups (P>0.05) . No patient occurred early death and relapse. The estimated 3-year overall survival (OS) of PNH and PNH-AA groups were (100.0±0.0) % and (85.7± 6.6) % (P=0.141) , GVHD-free and failure-free survival (GFFS) were (100.0±0.0) %, (78.7±7.7) % (P=0.067) . Conclusions allo-HSCT is effective for patients with PNH and PNH-AA syndrome. The preliminary results indicate that myeloid and platelet engraftment in PNH group were faster than PNH-AA group. There were no differences in OS and GFFS between PNH group and PNH-AA group.

Objective: To explore the prevalences of JAK2, CALR and MPL gene mutations and the mutation types in patients with Philadelphia chromosome negative myeloproliferative neoplasms (MPNs) , and to compare their clinical characteristics of different mutation types with each other and mutation negative group. Methods: The mutations of JAK2 V617F, JAK2 gene at exon 12, CALR gene at exon 9 and MPL gene at exon 10 in 1 648 Ph negative MPNs patients were detected by direct sequencing. Results: ① The JAK2V617F mutation was found in 471 (92.7%) of 508 PV patients, 819 (78.1%) of 1 049 ET patients and 74 (81.3%) of 91 PMF patients respectively, with the total mutation rate as 82.8% (1 364/1 648) . The JAK2 exon12 mutation was found in 9 (1.7%) of 508 PV patients, none was found in ET or PMF patients, with the total mutation rate as 0.5% (9/1 648) . The CALR mutation was found in 132 (12.6%) of 1 049 ET patients and 11 (12.1%) of 91 PMF patients respectively, with the total mutation rate as 8.7% (143/1 648) ; the MPL mutation was found in 9 (0.9%) of 1 049 ET patients and 1 (1.1%) of 91 PMF patients respectively, with the total mutation rate as 0.6% (10/1 648) . The co-occurrence of any two types of driver gene mutations was not detected by direct sequencing. ②The median onset age of patients with JAK2V617F[61 (15-95) y] was significant higher than of with JAK2 exon12 mutation[49 (33-62) y] or without mutations[42 (3-78) y] (P<0.001) , but not for patients with CALR[57 (17-89) y] or MPL mutation[59 (22-71) y] (P>0.05) . Patients with JAK2V617F had higher white blood cell count and hemoglobin level (P<0.05) when compared with patients with CALR mutation or without mutations, or only significantly higher white blood cell count when compared with patients with MPL mutation (P=0.013) . The platelet count of patients with CALR mutation was significantly higher than of with JAK2V617F[966 (400-2 069) ×10⁹/L vs 800 (198-3 730) ×10⁹/L, P<0.001]. ③Karyotype analysis was conducted in 1 160 patients with MPNs, the rates of karyotype abnormality of patients with and without CALR mutation were 9.8% (8/82) and 7.4% (80/1 078) (P=0.441) respectively; The rates of karyotype abnormality of patients with and without JAK2V617F mutation were 7.7% (75/971) and 6.9% (13/189) (P=0.688) respectively. The incidence of karyotype abnormality of patients with CALR mutation was higher than of with JAK2V617F[9.8% (8/82) vs 7.7% (75/971) ] without statistically significant difference (P=0.512) . The karyotype analysis of 7 cases of JAK2 exon12 mutation and 6 ones with MPL gene mutation revealed normal karyotype. Conclusions Driver gene mutations detection could ensure the diagnosis and prognosis judgment of MPN more reliable, different subtypes of MPNs had different profiles of driver gene mutations, the latter lead to unique clinical phenotype.

From October 2021 to February 2023, we retrospectively analyzed the clinical and laboratory data of six patients (three male and three female, median age: 54 years, age range: 29-73 years) with mast cell leukemia (MCL) diagnosed in the First Affiliated Hospital of Soochow University (The Mastocytosis Collaborative Network of China). All patients had acute MCL, with at least one C-finding present. The main clinical presentations were hypoalbuminemia ( n=4), fatigue ( n=3), fever ( n=2), abdominal discomfort ( n=2), osteolytic lesions ( n=2), dizziness ( n=1), skin flushing ( n=1), and weight loss ( n=1). Splenomegaly and lymphadenopathy were noted in six and three patients, respectively. Six patients were strongly positive for CD117, five were positive for CD30 and CD25, and four were positive for CD2. Four patients had a normal karyotype and two patients had an abnormal karyotype. Gene mutations were detected in 4/6 cases. The median serum tryptase level was 24.9 (range: 20.1-171.9) μg/L. Two patients were treated with venetoclax and azacitidine for induction (one patient achieved partial remission by combination with afatinib, while there was no remission after combination with dasatinib in the other patient). Two patients did not achieve complete remission despite treatment with cladribine and imatinib, respectively. One patient treated with interferon combined with glucocorticoids was lost to follow-up, and one patient abandoned treatment. The follow-up time ranged from 1.1 to 21.7 months. Three patients died and two survived. Overall, MCL is a rare subtype of systemic mastocytosis with heterogeneous clinical course, and these patients have poor outcome. A better understanding of the clinical characteristics, treatment, and prognosis of MCL is urgently needed.

Objective:To summarize the clinical characteristics of an early death in patients with de novo acute promyelocytic leukemia (APL) , analyze the risk factors and direct causes of early death, and perform survival analysis.Methods:The clinical data of 368 patients with de novo APL in three centers (First Affiliated Hospital of Soochow University, Soochow Guangci Hospital, and Soochow Hopes Hospital of Hematology) during January 2011-December 2017 were retrospectively analyzed. The clinical characteristics of patients who suffered hemorrhagic early death and non-hemorrhagic early death were compared. The risk factors for early death, survival, and prognosis of patients with APL were analyzed.Results:Among the 368 de novo APL patients, 31 died early with an early mortality rate of 8.4%. The median time from diagnosis to death was 7 (0-29) d. On comparison of the clinical characteristics of patients with early death and non-early death and subsequent multivariate analysis using a logistic regression model, it was observed that age ≥50 years and WBC ≥10×10 9/L were independent risk factors for early death ( P<0.01) . A total of 27 (87.1%) of the 31 early deaths was directly attributed to hemorrhage as the immediate cause of early death. Hemorrhage was the only cause of death in patients <50 years old and the major cause of death in patients ≥50 years old. A comparison of the clinical characteristics of patients with hemorrhagic early death and patients with non-hemorrhagic early death suggested that the median age and indirect bilirubin concentration of patients with hemorrhagic early death were lower than those with non-hemorrhagic early death ( P<0.05) . The median follow-up time for all patients was 41.0 (0.3-101.4) months. The 2-year overall survival (OS) rate was (93.5±1.3) %, and the 5-year OS rate was (91.0±1.5) %. The 2-year disease-free survival (DFS) rate was (98.8±0.6) %, and the 5-year DFS rate was (97.1±0.9) %. The 2-year OS rate of patients ≥50 years old and patients <50 years old was 79.3% vs 94.2%, P=0.000; the 2-year DFS rate was 92.3% vs 98.1%, P=0.023. The respective 2-year OS rates of high-risk and non-high-risk patients were 77.3% and 96.7% ( P=0.000) and the respective 2-year DFS rates were 94.0% and 98.4% ( P=0.139) . Conclusion:Age and WBC are independent prognostic factors for early death. We observed a difference in early mortality between high-risk and low-risk APL, but no difference in DFS rate.