Objective To explore the effect of amifostine combined with low-dose cyclosporine in treatment of refractory immune thrombocytopenia effect and related mechanisms.Methods 60 cases of refractory immune thrombocytopenia patients using parallel randomized controlled groups, divided into three groups, 20 cases in each group, amifostine group were treated with amifostine, cyclosporine group were treated with cyclosporine, amifostine+CSA group received amifostine+cyclosporine A treatment.The platelet count, platelet membrane glycoprotein antibody, lymphocyte subsets and bone marrow megakaryocyte count were observed and compared.Results After different treatment of three, six months, the level of platelet count of patients in three groups were compared with the group before treatment were significantly increased, and the treatment of platelet count level of amifostine group and cyclosporine group were significantly lower than that of amifostine +CSA group, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.The total efficacy of amifostine+CSA group was significantly higher than the other two groups, the difference was statistically significant ( P<0.05 ) , there was no significant difference between amifostine group and cyclosporine group.After the treatment, the platelet membrane glycoprotein GPIIb/IIIa antibody levels in three groups were significantly increased, and ring the detection level of amifostine+CSA group after treatment was significantly higher than the other two groups, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.After treatment, the three groups of CD4 +, CD4 +/CD25 +and CD4 +/CD8 +levels were significantly increased, CD8 +decreased significantly, the difference was statistically significant (P<0.05).And the level of change after treatment with amifostine +cyclosporine group was significantly higher than that of the other two groups, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.After treatment, the number of bone marrow megakaryocytes in the three groups was significantly lower than that before treatment , the level of count after treatment with amifostine +cyclosporine was significantly lower than that of the other two groups, the difference was statistically significant (P<0.05).there was no significant difference between amifostine group and cyclosporine group.The adverse reactions of amifostine group and amifostine+CSA group were significantly lower than that in cyclosporine group, the difference was statistically significant (P<0.05).there was no significant difference between amifostine group and amifostine+CSA group.Conclusion Amifostine combined with low dose of cyclosporine in treatment of refractory immune thrombocytopenia can play a synergistic effect, improve the therapeutic effect, and effectively reduce the dosage and adverse reactions.

Objective To investigate the clinicopathologic characteristics and prognosis of medullary breast carcinoma.Methods We conducted a retrospective analysis on clinical and pathologic data of 166 patients with medullary breast cancer.Results All the patients were female with a median age of 52 years old.The proportion of patients with stage Ⅰ,Ⅱ and Ⅲ disease was 16.9％,68.1％,15.0％,respectively.The Luminal,HER-2 overexpressing and triple-negative subtypes constituted 31.3％,8.4％,and 60.3％,respectively.There was significant difference in regional lymph node status of medullary breast cancer patients with different molecular types (x2 =18.248,P =0.003),but not in tumor size,TNM stage,histological grade,and expression of Ki67 (all P ＞ 0.05).Multivariate survival analysis indicated that TNM stage was an independent predictor in the prognosis of medullary breast cancer (HR =5.664,P =0.001).All the patients were followed up from 15 months to 145 months with a median follow-up time of 108 months.The 5-year survival rate was 91.5％ and the 10-year survival rate was 87.2％.Conclusions The prognosis of medullary breast cancer is favorable.Personalized treatment according to the TNM stage and histopathologic characteristics achieve a favorable prognosis.

Objective To explore the clinicopathological characteristics and prognosis of invasive micropapillary carcinoma of the breast (IMPC),and the distinction between IMPC and invasive ductal carcinoma of the breast (IDC).Methods From February 2004 to November 2013,195 IMPC patients and 420 IDC patients were analyzed retrospectively.Results There were significant differences in mammilla invasion,lymph vessel invasion,orange peel sign,soft tissue encroachment,neoadjuvant chemotherapy,radical mastcctomy,lymph node metastasis,clinical stages,tumor size,lymph node staging,estrogen receptor (ER),progestin receptor (PR),human epidermal growth factor receptor 2 (HER2),molecular subtyping,ratio of radiation,ratio of endocrine therapy,disease-free survival (DFS),overall survival (OS)between the two groups,all P ＜0.05.Patients with IMPC had lower 5-year DFS and OS rates (68.2％ and 73.8％,respectively) than IDC patients (85.7％ and 88.6％,respectively),all P ＜ 0.05.In IMPC patients with positive ER/PR,HER2-negative,smaller tumor volume,less lymph node metastasis,negative nipple invasion,negative lymphatic vessel tumor thrombus,negative orange peel change had higher 5-year DFS and OS rates than those with negative ER/PR,HER2 overexpression,larger tumor volume,more lymph node metastasis,positive nipple invasion,positive lymphatic vessel tumor thrombus,positive orange peel change,all P ＜ 0.05.Besides,the patients with pathologic stage Ⅰ had higher OS than those with stage Ⅲ (P ＜ 0.05).Cox regression analysis found that orange peel change,lymph vessel invasion and HER2 were the independent risk factors for the survival time of patients with IMPC.Conclusions IMPC patients have lower DFS and OS compared with IDC.

AIM: To investigate the regulatory function of bone marrow-derived mesenchymal stem cells (MSCs) on T helper 17 cells (Th17) and regulatory T cells (Treg) in peripheral blood of severe asthmatic children . METHODS:MSCs were isolated , cultured and identified in vitro.MSCs digested with mitomycin were cocultured with T lymphocytes (TLC) at different ratios (1∶1, 1∶2, 1∶10 and 1∶20) from severe asthmatic children for 72 h.The prolifera-tion of TLC was measured by CCK-8 method.In the coculture system of the 1∶2 ratio and the single TLC system , the super-natant levels of interleukin-17 (IL-17) and transforming growth factor-β(TGF-β) were measured by ELISA.The mRNA expression of retinoic acid-related orphan nuclear receptor C (RORC) and forkhead box protein 3 (Foxp3) in TLC was de-tected by qRT-PCR.RESULTS:After cocultured with MSCs , the proliferation of TLC decreased significantly in a dose-dependent manner (P<0.05).It also showed decreases in IL-17 (3 799 ±441 vs 4 890 ±373, P<0.05) and RORC mRNA level (1.21 ±0.14 vs 3.85 ±0.48, P<0.05), while an increase in TGF-βlevel (209 ±32 vs 117 ±26, P<0.05) was observed.No influence on the mRNA expression of Foxp3 was found (P>0.05).CONCLUSION: MSCs suppresses Th17 polarization of naive peripheral blood CD 4 +T cells and matures Th17 cells secreting IL-17, which may ef-fectively revise Th17/Treg imbalance of asthma .

Objective To reproduce an SD rat model of prostatic calculus by using nanobacteria (NB), and explore the role of NB in contributing to prostatitis and prostatic calculus. Methods Twenty adult male SD rats were randomized to the control group and 20 to the model group. Rat prostate infection models were reproduced by infusing 0. 2 ml (Concentration, 1 Mai unit) NB suspension transurethrally. 0.2 ml physiological saline was infused transurethrally in the rat control group. The rats were sacrificed 4 and 8 weeks later and prostatic pathology were viewed by hematoxylin and eosin (HE) staining. Lithogenesis was observed by scanning electron microscope (SEM) or Transmission electron microscopy (TEM). Re-isolation, culture and identification of nanobacteria were also done in rat prostatic tissues. Results Chronic inflammatory changes of prostates were shown in the model group at both 4 weeks and 8 weeks after infusing NB suspension. Prostatic calculi were detected by SEM and TEM at 8 weeks in the prostates of the rat model group (7/10). Neither chronic inflammatory changes nor prostatic calculus was found in the control group. NB was positive in the model group, but negative in the control group. Conclusions NB infection could cause chronic prostatitis and prostatic calculus in rats.

Objective To investigate the clinical and pathologic features of squamous cell carcinoma of the breast.Methods The clinical and pathologic data of 23 squamous cell carcinoma of the breast patients admitted between 1984 and 2013 to Tianjin Medical University Cancer Hospital was analyzed retrospectively.Results Primary squamous cell carcinoma of the breast was a very rare tumor accounting for 0.06％ of all breast cancers.All of the 23 patients were females aged 28 years to 87 years(median age 49 years).Average tumor size was 4.5 cm.9 patients suffered from lymph node metastasis at admission (39.1％).The positive rates of estrogen receptor (ER),progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER-2) were 13.6％ (3/23),4.5％ (1/23) and 0 (0/20) respectively.With a follow-up time varying from 5 months to 36 months recurrence or metastasis were found in 8 patients,and another 1 patient was found having distant metastasis at admission.Lung metastasis (7/9) was most common.6 patients died.Conclusions Squamous cell carcinoma of the breast is highly invasive,with low rate of positive receptors and early distant metastasis or recurrence after operation,and poor patients' survival.

Objective To explore the diagnostic value of protein induced by vitamin K absence or antagonist-Ⅱ (PIVKA-Ⅱ) in non-infant with acquired deficiency of vitamin K-dependent coagulation factors (ADVKCF).Methods PIVKA-Ⅱ levels were measured by ELISA in 50 patients with ADVKCF on day 0,3,7 after vitamin K treatment.Prothrombin time(PT),APTT,FⅡ ∶ C,FⅦ∶ C,FⅨ∶ C,and FⅩ∶ C were analyzed simultaneously.Twenty healthy subjects were enrolled as controls.Results The average level of PIVKA-Ⅱ in ADVKCF group was (3.83 ± 1.40) μg/L,while (1.30 ± 0.54) μg/L in the control group (P ＜ 0.05).The PIVKA-Ⅱ levels on day 0 and 3 did not show significant difference [(3.83 ± 1.40) μg/Lvs (3.79 ± 0.66) μg/L,P ＞ 0.05],but decreasing significantly on day 7 compared to the control group (P ＜ 0.05).The PIVKA-Ⅱ level was (3.78 ± 1.30) μg/L in patients receiving plasma transfusion,while (3.91 ± 1.49)μg/L in no-plasma-transfusion group (P ＞ 0.05).Coagulation factors Ⅱ,Ⅶ,Ⅸ and Ⅹ activity which decreased significantly before treatment returned to normal range after one week use of vitamin K,leading to complete correction of prolonged APTT and PT (＞ 100 seconds).Conclusions The PIVKAⅡ level in ADVKCF patients is significantly higher than that of healthy subjects within one week treatment of vitamin K,which is not influenced by plasma transfusion.This study suggests that PIVKA-Ⅱ is a more sensitive parameter than APTT,PT and the activity of coagulation factor,which could be a valuable factor in the early diagnosis of ADVKCF.

Objective To investigate the clinical pathological characteristics, diagnosis and treatment of breast rhabdomyosarcoma, and to enhance the awareness of malignancy infiltration to bone marrow (BM). Methods The data of one case of Rhabdomyosarcoma of breast were analyzed retrospectively. BM aspirate and biopsy, morphology, immunology, cytogenetics, molecular biology (MICM) in different parts of BM, peripheral blood smear, fine puncture of breast mass, final biopsy of breast mass by Mammotome System and whole body PET-CT were performed. The immunochemistry stain of specimen of breast mass was used. Results The peripheral blood smear of this patient showed immature erythrocytes, leucocytes and classification of unknown cells which were consistent with BM morphology. The results of BM aspirate and biopsy depicted a hypercellular specimen with disseminated unknown cells infiltration. Unknown cells were positive for CD56 and negative for any hematopoietic markers by flow cytometry. The whole body PET-CT showed that uptake of 18F-FDG of bilateral breast and whole BM was increased, whereas the mass of breast was not presented by CT. PET-CT suggested a probable malignant hematologic disease. The enough specimen of breast mass got from Mammotome System showed embryonal rhabdomyosarcoma, and the tumor cells were positive for MyoD1, Vimentin and Desmin. Conclusions It is a challenge for early diagnosis of solid sarcoma with unknown origin which diffusely infiltrating into BM. Negative expression of hematopoietic markers by flow cytometry plays a role on differential diagnosis in this setting, whereas PET-CT only provides a valuable reference. Enough specimen and immunohistochemical staining could provide solid evidences of diagnosis.

Objective To explore the clinical features and causes of misdiagnosis of the patients with acquired deficiency of vitamin K-dependent coagulation factors (ADVKDCF). Methods Retrospective analysis was performed with the data from 62 patients with ADVKDCF for etiological factors, clinical manifestations,laboratory examinations, diagnosis and treatments. Results Among the 62 patients, 51 patients were with unknown causes( subgroup A) and 11 were with clear histories of anticoagulant rodenticide poisoning( subgroup B). The presentations of hemorrhage of the patients varied with hematuria as the most common first symptom,followed by skin, mucosa, muscle, internal organs bleeding (28/62). The most common hemorrhage symptom is hematuria. 35 of the 62 patients had hemoglobin(Hb) levels less than 100 g/L due to blood loss( the lowest level was 32 g/L). Thirty-eight patients were misdiagnosed at the first visit and the median time from hemorrhage manifestation to definite diagnosis was 8 days (range,2 to 192 days). ADVKDCF was mostly misdiagnosed as the urinary system diseases (23/38), followed by hemophilia (8/38). Laboratory examinations showed normal platelet count , throm bin time (TT) and normal fibrinogen(Fg) concentration, but prolonged plasma prothrombin time (PT), activated partial prothrombin time (APTT) and international normalized ration (INR). All of patients received high dose vitamin K ( intravenous vitamin K1 with a initial dose of 20 to 240 mg/d and then oral vitamin K4 maintenance) . The bleeding symptoms disappeared 1 day after treatment and the Hb levels increased dramatically. There were significant differences in PT, APTT and INR of the patients before and after treatment( P ＜0. 01 ). Followed by a median follow - up of 8 months , no patient had severe adverse effects or recurrence. Conclusion The hemorrhage presentations of the patients with ADVKDCF are various. The most common hemorrhage symptom is hematuria. The misdiagnosis rate of ADVKDCF is high with urinary systems disorders as the most common misdiagnosis. Sequential treatment with vitamin K is an effective and safe method to prevent recurrence. Early detection of coagulation function is helpful to reduce misdiagnosis possibility.

OBJECTIVE: To explore the genetic etiology for a newborn with corneal opacity. METHODS: The neonate and her parents were subjected to routine G-banding chromosomal karyotyping analysis. Copy number variation (CNV) was analyzed with low-coverage whole-genome sequencing (WGS) and single nucleotide polymorphism microarray (SNP array). RESULTS: No karyotypic abnormality was found in the newborn and her parents. Low-coverage WGS has identified a de novo 5.5 Mb microdeletion at chromosome 8q21.11-q21.13 in the neonate, which encompassed the ZFHX4 and PEX2 genes. The result was confirmed by SNP array-based CNV analysis. CONCLUSION The newborn was diagnosed with chromosome 8q21.11 deletion syndrome. ZFHX4 may be one of the key genes underlying this syndrome.
Chromosome Banding ; Chromosomes, Human, Pair 8/genetics* ; DNA Copy Number Variations ; Female ; Genetic Testing ; Homeodomain Proteins/genetics* ; Humans ; Infant, Newborn ; Karyotyping ; Monosomy/genetics* ; Peroxisomal Biogenesis Factor 2/genetics* ; Polymorphism, Single Nucleotide ; Transcription Factors/genetics*