Adult suspected primary hemophagocytic syndrome complicated with aggressive natural killer cell leukemia: report of one case and review of literature / 白血病·淋巴瘤

Objective:To improve the understanding of adult primary hemophagocytic syndrome (HPS) with aggressive natural killer cell leukemia (ANKL).Methods:The clinicopathological data of one adult patient with suspected primary HPS complicated with ANKL in Huiqiao Medical Center, Nanfang Hospital of Southern Medical University in October 2017 were retrospectively analyzed, and literatures were reviewed.Results:A 21-year-old male patient presented with persistent fever, hemocytopenia, splenomegaly, low fibrinogen, a significant increase in ferritin, hemophagocytes in bone marrow, decreased natural killer (NK) cell activity, and increased soluble CD25. Flow cytometry detection showed that the expression of NK cells was abnormal, and there were familial lysosomal trafficking regulator (LYST) and UNC13D gene defects. He was suspected of primary HPS complicated with ANKL. The patient was given 4 courses of EPOCH+PEG-Asp (etoposide, dexamethasone, vindesine, cyclophosphamide, doxorubicin hydrochloride liposome, pegaspargase) regimen chemotherapy, 20 mg of citalopidine twice a week maintenance therapy and matched unrelated hematopoietic stem cell transplantation. After 35 months of follow-up, he got sustained remission.Conclusions:Even if there are secondary causes of adult HPS, it is necessary to screen out related genes to avoid misdiagnosis. HPS patients with ANKL progress rapidly, and the early mortality is high. EPOCH+ PEG-Asp regimen induction therapy and allogeneic hematopoietic stem cell transplantation should be used as early as possible after diagnosis.

Clinical features and imaging diagnosis of tuberous sclerosis / 西安交通大学学报(医学版)

【Objective】 To explore the clinical features and the imaging diagnosis value of tuberous sclerosis. 【Methods】 We retrospectively analyzed the clinical data and imaging findings of 22 patients with tuberous sclerosis clinically diagnosed in our hospital. 【Results】 There were 12 male and 10 female patients, with the mean age of 7.3 years. Among them, 2 patients had a family history; 3 had unclear symptoms and the rest 19 ones except 1 had facial skin rashes for two years, which led to seek for medical help, and the other 18 had epilepsy attack, including 4 with mental retardation. All the 22 patients had characteristic subependymal nodules, including 21 patients with multiple abnormal changes in the bilateral cerebral hemispheres. There were 12 cases with abnormal skin appearance, 2 cases accompanied by facial cortical adenoma, 1 case with right subependymal giant cell astrocytoma, 1 case with bilateral ependymoma, 1 case with Dandy-Wallker malformation, 1 case with right renal duplication, 2 cases with renal hamartoma, 1 case with multiple renal nodules, 1 case with congenital patent ductus arteriosus, 2 cases with cardiac rhabdomyosarcoma, 1 case with multiple hepatic hamartoma, and 2 cases with multiple sclerotin sclerosis changes. 【Conclusion】 The clinical manifestations of tuberous sclerosis are complex and variable, and there are certain imaging characteristics associated with multi-organ involvement. Familiarity with the characteristics and imaging manifestations of lesions in various systems can make the clinical diagnosis more concise and accurate.

Selection and appraisement of nucleic acid aptamers for castration resistant prostate cancer cells based on Cell-SELEX / 西安交通大学学报(医学版)

【Objective】 To screen nucleic acid aptamers that can specifically recognize castration-resistant prostate cancer （CRPC） cells by Cell-SELEX. 【Methods】 For Cell-SELEX selection， CRPC cell lines C4-2 were used as positive control cells， while androgen-dependent prostate cancer cell lines LNCap were used as negative control cells. The 5’end of the upstream primers was labeled with FITC， and the 5’end of the downstream primers was labeled with biotin. Single-stranded DNA （ssDNA） collected from each round of screening was used as template for PCR amplification and purification. The biotin-streptavidin magnetic bead separation was used to isolate PCR product for the next round of screening. The process of Cell-SELEX was analyzed by flow cytometry. ssDNA products of the last round were collected for PCR amplification， purification， cloning and DNA sequencing. The secondary structure of selected DNA aptamers was predicted. Dissociation constants of the two aptamers were calculated. Flow cytometry and confocal microscopy were used to evaluate selective binding of aptamers to CRPC cells and tissues. 【Results】 The binding rate of DNA products to CRPC cells gradually increased with the increase of selection cycles， reaching the highest in the last round. DNA structure prediction analysis showed that the secondary structure of aptamers CRPC-1 and CRPC-2 was mainly stem-loop structure. Flow cytometry analysis and confocal images showed that both CRPC-1 and CRPC-2 could specifically target C4-2 cells. In addition， immunohistofluorescence assay showed that CRPC-1 could specifically target CRPC tissues. 【Conclusion】 Cell-SELEX can be used to screen aptamers that specifically target CRPC cells and tissues， which provides experimental basis for early screening and targeted diagnosis of CRPC. It is of significance for treatment plan adjustment and prognosis improvement of prostate cancer.

Clinical features of dermatomyositis and MRI diagnostic value / 西安交通大学学报(医学版)

【Objective】 To explore the clinical features of dermatomyositis and diagnostic value of MRI. 【Methods】 The clinical and imaging data of 61 patients with dermatomyositis were analyzed retrospectively. 【Results】 Among the 61 patients， there were 15 males and 46 females with the average age of 45.3 years. The course of disease was 10 days to 12 years. All of them had different degrees of skin lesions， including 57 cases of myasthenia and or myalgia， 4 cases of dysphagia， 2 cases of dyspnea， and 2 cases of drinking water and coughing. There were 18 cases complicated with interstitial lung disease， 1 case complicated with Sjogren’s syndrome， 1 case complicated with arthritis， 1 case complicated with rhabdomyolysis， and 1 case complicated with kidney damage and liver insufficiency. MRI examination performed in 46 patients with dermatomyositis detected 43 cases of abnormal manifestations， 41 cases of muscle inflammatory edema， 37 cases of subcutaneous adipose tissue edema， and 40 cases of myofascitis. 【Conclusion】 The clinical manifestations of dermatomyositis are complex and have certain imaging features. Combined with the laboratory examinations of muscle enzymes and autoantibodies， electromyography， skin and muscle biopsy， they can provide clinical diagnostic evidence. MRI is an important means to diagnose and evaluate the extent and progression of dermatomyositis.

Comparison of Urologist Satisfaction for Different Types of Prostate MRI Reports: A Large Sample Investigation

Objective@#To evaluate urologist satisfaction on structured prostate MRI reports, including report with tumor-node-metastasis (TNM) staging (report B) and with Prostate Imaging Reporting and Data System (PI-RADS) score with/without TNM staging (report C, report with PI-RADS score only [report C-a] and report with PI-RADS score and TNM staging [C-b]) compared with conventional free-text report (report A). @*Materials and Methods@#This was a prospective comparative study. Altogether, 3015 prostate MRI reports including reports A, B, C-a, and C-b were rated by 13 urologists using a 5-point Likert Scale. A questionnaire was used to assess urologist satisfaction based on the following parameters: correctness, practicality, and urologist subjectivity. Kruskal-Wallis H-test followed by Nemenyi test was used to compare urologists’ satisfaction parameters for each report type. The rate of urologist-radiologist recalls for each report type was calculated. @*Results@#Reports B and C including its subtypes had higher ratings of satisfaction than report A for overall satisfaction degree, and parameters of correctness, practicality, and subjectivity (p 0.05). Compared with report C-b (p > 0.05), report B and C-a (p 0.05). No statistical difference was found between report C-a and C-b in overall satisfaction degree and all three parameters (p > 0.05). The rate of urologist-radiologist recalls for reports A, B, C-a and C-b were 29.1%, 10.8%, 18.1% and 11.2%, respectively. @*Conclusion@#Structured reports, either using TNM or PI-RADS are highly preferred over conventional free-text reports and lead to fewer report-related post-hoc inquiries from urologists.

Comparison of Urologist Satisfaction for Different Types of Prostate MRI Reports: A Large Sample Investigation

Objective@#To evaluate urologist satisfaction on structured prostate MRI reports, including report with tumor-node-metastasis (TNM) staging (report B) and with Prostate Imaging Reporting and Data System (PI-RADS) score with/without TNM staging (report C, report with PI-RADS score only [report C-a] and report with PI-RADS score and TNM staging [C-b]) compared with conventional free-text report (report A). @*Materials and Methods@#This was a prospective comparative study. Altogether, 3015 prostate MRI reports including reports A, B, C-a, and C-b were rated by 13 urologists using a 5-point Likert Scale. A questionnaire was used to assess urologist satisfaction based on the following parameters: correctness, practicality, and urologist subjectivity. Kruskal-Wallis H-test followed by Nemenyi test was used to compare urologists’ satisfaction parameters for each report type. The rate of urologist-radiologist recalls for each report type was calculated. @*Results@#Reports B and C including its subtypes had higher ratings of satisfaction than report A for overall satisfaction degree, and parameters of correctness, practicality, and subjectivity (p 0.05). Compared with report C-b (p > 0.05), report B and C-a (p 0.05). No statistical difference was found between report C-a and C-b in overall satisfaction degree and all three parameters (p > 0.05). The rate of urologist-radiologist recalls for reports A, B, C-a and C-b were 29.1%, 10.8%, 18.1% and 11.2%, respectively. @*Conclusion@#Structured reports, either using TNM or PI-RADS are highly preferred over conventional free-text reports and lead to fewer report-related post-hoc inquiries from urologists.

SELEX screening of EpCAMGspecific nucleic acid aptamer targeting prostate cancer cells / 实用放射学杂志

Objective To investigate the methods of screening specific aptamers for (EpCAM)Gpositive prostate cancer (PCa)cells by cellGSELEX technique.Methods A random DNA library was designed to screen EpCAMGspecific DNA aptamers from human prostate cancer cells expressing EpCAM molecule by cellGSELEX technique.After 12 rounds of in vitro screening,DNA products were cloned and sequenced.Flow cytometry and cellular immunofluorescence were used to detect the specific binding ability of aptamers to target cells.Results Two aptamers of Ep1 and Ep2 were selected.Both of them could specifically bind to EpCAMGpositive cancer cells LNCap,PCG3 ,DU1 45 , and HEK293T cells transfected with target molecule.The binding rates of Ep1 were 61.0%,74.3%,5 9.1% and 60.3%.The binding rates of Ep2 were 65.1%,77.8%,54.2% and 58.3%.Neither of them could bind to HEK293T cells transfected with empty vector with the binding rate of 5.4% in Ep1 and 3.3% in Ep2,respectively.Flow cytometry analysis and confocal images indicated that the EpCAM aptamers could specifically recognize human PCa cells expressing EpCAM,but could not bind to EpCAMGnegative cells.Conclusion EpCAM aptamers derived from cellGSELEX technology can recognize and bind to EpCAMGpositive PCa cells specifically,which may provide new ideas for the specific diagnosis and targeted therapy of prostate cancer,and lay an experimental basis for the other specific diagnosis and treatment schemes of malignant tumors.

The application value of monoexponential,biexponential models multiple b values DWI in pancreatic cancer / 实用放射学杂志

Objective To explore the application value of monoexponential, biexponential models multiple b values diffusion weighted imaging(DWI) in distinguishing pancreatic cancer from non-tumorous pancreas.Methods Subjects comprised 37 pancreatic cancers confirmed by clinical or surgery.Pancreas multiple b values DWI was performed using 3.0T scanner.Standard apparent diffusion coefficient (ADCstandard) was calculated using monoexponential diffusion model.Pure diffusion coefficient (ADCslow), pseudodiffusion coefficient (ADCfast) and perfusion fraction (f) were calculated using intravoxel incoherent motion(IVIM) diffusion model.Parameters of pancreatic cancers and non-tumorous pancreas were compared using independent samples t test.Results Mean ADCslow value of pancreatic cancer was higher than that of non-tumorous pancreas (0.611×10-3 mm2/s vs 0.521×10-3 mm2/s,P=0.037).Mean ADCfast and f values of pancreatic cancer were lower than that of non-tumorous pancreas (5.066×10-3 mm2/s vs 7.188×10-3 mm2/s,P=0.035;55.8% vs 64.0%,P=0.016;respectively).ADCslow of pancreatic cancer was positively correlated to ADCstandard (r=0.824,P=0.000).ADCfast of pancreatic cancer was negatively correlated to f(r=-0.558,P=0.000).Conclusion ADCslow, ADCfast and f derived from IVIM-DWI model can distinguish pancreatic cancer from non-tumorous pancreas.IVIM-DWI may be a promising and non-invasive tool for early diagnosing and differentiating pancreatic carcinoma from non-tumorous pancreas.

Analysis of CT fingings and the causes of missed diagnosis of insulinoma / 实用放射学杂志

Objective To analyze CT findings of insulinoma,and to summarize the causes of missed diagnosis of nontypical insuli-noma.Methods Clinical and CT manifestations of 18 patients with 18 insulinomas were analyzed retrospectively which were proved by surgery and pathology,and the causes of the missed diagnosis of nontypical insulinoma were also summarized.Results 10 patients with 10 insulinoma underwent CT plain scan with isodensity in 9 and slightly lower density in 1 with thread-like capsule.Other 18 patients underwent enhanced CT scan,10 of whom showed obvious enhancement in arterial phase with isodentisy in 6 and slightly higher density in 4 in portal phase,and isodensity in 10 in delayed phase.4 lesions showed mild-to-moderate enhancement in arterial phase with slightly higher density than normal pancreas in 2 and isodentisy in 2 in portal phase,and slightly higher density than nor-mal pancreas in 1 and similar density to pancreas in 3 in delayed phase.In portal phase,the enhanced degree in 8 was similar to the pancreas,and that in 6 was slightly higher or higher than that of pancreas.In delayed phase,13 were similar to the pancreas and other 1 was higher than that.3 of 18 lesions were easily missed,and 4 lesions with missed diagnosis showed isodensity on plain CT and en-hanced CT,and were further detected by other imaging methods.Conclusion Multiphase enhancement CT scanning can be used as the first choice for the insulinoma.