Objective To study the therapeutic effect of adjuvant chemotherapy and radiotherapy after operation in patients with mucoepidermoid carcinoma of parotid gland,and to screen the indicators ralated to the prognosis of tumor.Methods Eighty patients with mucoepidermoid carcinoma of parotid gland in First People′Hospital of Yibin of Sichuan Province from January 2005 to December 2009 were analysed retrospectively in our research.We studied the survival of patients who were treated wtih simple operation(30 cases)or postoperative adjuvant therapy(50 cases).Then we further analyzed the relationships between the prognosis of the patients and some variables (age,gender,smoking,alcohol drinking,lymph node metastasis,distant organ metastasis,treat-ment method,differentiation degree and T grading).Results Kaplan-Meier survival curves showed that patients with postoperative adjuvant therapy had longer PFS and OS than those without adjuvant therapy (94.4 months vs 69.3 months;114.9 months vs 96.7 months),with statistical significance (χ2 =11 .246,P =0.001 ;χ2 =15.803,P =0.001 ).COX univariate analysis showed that gender (χ2 =22.346,P =0.000),smoking (χ2 =7.891 ,P =0.041 ),lymph node metastasis (χ2 =12.371 ,P =0.005),distant organ metastasis (χ2 =9.81 3, P =0.002),treatment method (χ2 =25.261 ,P =0.000),differentiation degree (χ2 =4.361 ,P =0.006)and T grading (χ2 =5.336,P =0.01 4)were related to the PFS of patients.COX multivariate analysis showed that lymph node metastasis (χ2 =11 .003,RR =2.827,95%CI:1 .965-3.851 ,P =0.011 ),distant organ metastasis (χ2 =7.611 ,RR =0.472,95%CI:0.240-0.775,P =0.016),treatment method (χ2 =24.542,RR =5.390, 95%CI:3.585-9.602,P =0.000),degree of differentiation (χ2 =3.221 ,RR =2.1 1 8,95%CI:1 .845-4.719, P =0.009)and T grading (χ2 =4.336,RR =0.804,95%CI:0.681 -0.916,P =0.024)were related to the PFS of patients.COX univariate analysis showed that smoking (χ2 =4.551 ,P =0.008),alcohol drinking (χ2 =11 .742,P =0.048),lymph node metastasis (χ2 =14.886,P =0.009),distant organ metastasis (χ2 =6.71 3, P =0.005),treatment method (χ2 =22.411 ,P =0.000),degree of differentiation (χ2 =8.1 16,P =0.012)and T grading (χ2 =14.443,P =0.035)were related to the OS of patients.COX multivariate analysis showed that lymph node metastasis (χ2 =11 .711 ,RR =2.985,95%CI:1 .521 -3.999,P =0.005),distant organ metastasis (χ2 =5.390,RR =0.400,95%CI:0.201 -0.793,P =0.009),treatment method (χ2 =19.327,RR =5.086, 95%CI:3.241 -8.006,P =0.000),degree of differentiation (χ2 =7.084,RR =2.301 ,95%CI:1 .908-4.503, P =0.001 )and T grading (χ2 =1 3.229,RR =0.561 ,95%CI:0.348-0.867,P =0.040)were related to the OS of patients.Conclusion Adjuvant radiation and chemotherapy can obviously prolong the PFS and OS for the patients with mucoepidermoid carcinoma of parotid gland.Lymph node metastasis,distant organ metastasis,treat-ment method,differentiation degree and T grading can greatly influence the prognosis of patients with mucoepider-moid carcinoma of parotid gland,which can be used as independent prognostic indicators for the patients with mucoepidermoid carcinoma of parotid gland.

Objective To investigate the correct diagnosis and treatment of myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene. Methods A case of patient who was diagnosed as myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene was reported, and the literature was reviewed. Results The patient was diagnosed as typical T-lymphoblast lymphoma (T-LBL) by the lymph node pathologic diagnosis, while the diagnosis of myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene was made correctly by the whole examination and analysis. The patient acquired deep complete remission quickly after taking the low dose of imatinib. Conclusions Myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene are a rare hematologic tumor. Though pathological diagnosis is the golden standard for lymphoma, sometimes the other factors should be taken into consideration and make an overall analysis of clinical picture and a correct view of the pathological diagnosis, which could avoid the misdiagnosis and improper treatment.

Objective:To investigate the efficacy of new-type stereotaxic apparatus-assisted transfrontal puncture and drainage in the treatment of hypertensive intracerebral hemorrhage in the basal ganglia.Methods:A retrospective analysis was performed on the clinical data of 60 patients with hypertensive intracerebral hemorrhage in the basal ganglia who received disposable new-type stereotaxic apparatus-assisted transfrontal insertion with soft tunnels for hematoma aspiration drainage in our hospital from August 2017 to September 2019. The treatment efficacy was analyzed.Results:All patients were successfully punctured at one time; the puncture surface was 5-6.5 cm on the basement plane, where the hematoma surface was the largest; the puncture angle was 10-14°, and the puncture depth was 9-11.5 cm. Fifteen patients were operated within 6 h of hemorrhage, and the intraoperative hematoma clearance rate was about 25%; 40 patients were operated 6-24 h after hemorrhage, and the hematoma clearance rate was about 20%; 5 patients were operated one-3 d after hemorrhage, and the hematoma clearance rate was as high as 30%. The first postoperative re-check CT showed that 51 patients had ideal position of the drainage tube, 2 were too deep, one was too shallow, 2 were below the position, 2 were above the position, one was inside the position, and one was outside the position. The Glasgow Coma Scale (GOS) scores of the patients on 3 rd d of operation (9.88±3.998) were significantly higher than those of the patients before operation (6.24±3.159, P<0.05). One month after the operation, GOS showed that 20 patients (33.3%) had good recovery, 28 (46.7%) had mild disability, 7 (11.7%) had severe disability, 3 (5.0%) had plant survival, and 2 (3.3%) died. Conclusion:The disposable new-type stereotaxic apparatus-assisted transfrontal puncture drainage is easy to be conducted and practicable with a reasonable design, accurate positioning, minimal surgical traumas and satisfactory curative effect.

Objective: To evaluate the efficacy and tolerability of ruxolitinib combined with prednisone, thalidomide and danazol for treatment of in myelofibrosis (MF). Methods: Patients of MF according to the WHO 2016 criteria, received ruxolitinib (RUX) combined with prednisone, thalidomide and danazol (PTD). The response, changes of blood counts and adverse events were evaluated. Results: Six PMF and one post-ET MF patients were enrolled. Four patients presented JAK2V617F mutation, one CALR mutation, one MPL mutation, one triple-negative. Responses per IWG-MRT criteria were clinical improvement in 5 patients, stable disease in 2 ones, spleen response in 6 ones. All of 7 patients were symptomatic responses, four patients achieved at least 50% improvement from baseline on MPN-SAF TSS. Three patients initially treated with RUX alone, all of 3 patients experienced treatment-associated anemia and thrombocytopenia. Then these 3 patients received RUX combined with PTD, both hemoglobin and platelet increased significantly. Four patients initially treated with RUX combined with PTD. Increased levels of hemoglobin and platelet were seen in all of 7 patients received RUX combined with PTD with maximum increased hemoglobin of 30(18-54) g/L and maximum increased platelets of 116(13-369)×10⁹/L, respectively from baseline. The treatment dose of RUX increased due to improved platelet count in 3 patients. The frequent non-hematologic adverse events grade 1-2 were constipation, abdominal distension, crura edema and increased ALT. Conclusions RUX combined with PTD for treatment of MF may modulate initial hematologic toxicity observed when RUX alone, and may increase response due to improved levels of hemoglobin or platelet.

Objective: To observe the clinical efficacy and safety of the patients of myelodysplastic syndromes-refractory anemia with excess blasts (MDS-REAB) treated with decitabine alone or based on low dose cytarabine (Ara-C) regimen CAG/HAG [aclarubrci (ACR) /homoharring-tonine (HHT) +cytarabine+granulocyte colony stimulating factor (G-CSF) ]. Methods: Totally 121 patients with MDS-REAB were retrospectively analyzed, including 59 patients treated with decitabine alone (20 mg·m^-2·d^-1 for 5 days) , the rest 62 ones treated with low-dose Ara-C-based regimen CAG/HAG. Overall response rate (ORR) , overall survival (OS) and adverse events of the two groups were analyzed and compared retrospectively. Results: The ORR of decitabine alone or CAG/HAG were 66.2% and 56.4% respectively, with no statistically significant differences (χ²=1.185, P=0.276) . Initial response rate detected by the end of first cycle of CAG/HAG was higher than that of decitabine alone (94.3% vs 69.2%) , there was statistically significant difference in the overall comparison of two groups (χ²=7.612, P=0.009) . The median OS of decitabine alone was 19.5 (95% CI 10.5-28.4) months, the median OS of CAG/HAG was 20.3 (95% CI 10.7-29.9) months, with no statistically significant differences (χ²=0.004, P=0.947) . Grade 3-4 cytopenia and infection were the most prevalent adverses of two group patients. Grade 3-4 cytopenia rate of CAG/HAG was higher than that of decitabine alone (100.0% vs 64.4%, P<0.001) . The infection rate detected at third cycle of CAG/HAG was higher than that of decitabine alone (52.9% vs 15.2%, P=0.008) . Conclusion The efficacy of treating MDS-RAEB with decitabine alone or CAG/HAG was equivalent. CAG/HAG treatment came into effect faster, but decitabine alone treatment was safer.

Objective: To observe the clinical characteristics, treatment responses and prognosis of patients with myelodysplastic syndrome (MDS) -del (5q) syndrome who met WHO (2016) diagnostic typing criteria. Methods: A total of 77 patients with del (5q) syndrome, according to WHO (2016) classification, were retrospectively analyzed between January 2008 and April 2018 in the Blood Diseases Hospital, Chinese Academy of Medical Sciences. Clinical characteristics, lenalidomide (LEN) efficacy and survivals were compared between the patients with del (5q) alone and those with one additional cytogenetic abnormality (ACA) with the exception of monosomy 7 or del (7q) . Treatment response and overall survival (OS) were compared between patients who were treated with LEN and traditional non-LEN drugs. Results: Of 77 patients, 64 were isolated del (5q) and 13 were del (5q) with ACA. There were significant differences of the median age and percentage of patients who had small megakaryocytes in bone marrow smear by immunohistochemistry (CD41) between the patients with isolated del (5q) and the patients with del (5q) + ACA[58 (29-64) years old vs 63 (31-82) years old, z=2.164, P=0.030; and 91.7%vs 60.0%, P=0.046, respectively]. The overall hematological response rate (78.9%vs 80.0%) , complete hematological remission (CR) rate (57.9% vs 60.0%) , cytogenetic response (CyR) rate[69.2% (9/13) vs 66.7% (4/6) ] and complete cytogenetic response (CCyR) rate [61.5% (8/13) vs 33.3% (2/6) ] of LEN were similar between the patients with isolated del (5q) (n=19) and with del (5q) + ACA (n=10) , as well as the median Overall survival (OS) between these two groups of patients (62 months vs 78 months, P=0.388) . The hematological response rate (79.3% vs 36.0%) , CR rate (58.6% vs 8.0%) , CyR rate [68.4% (13/19) vs 11.1% (1/9) ] and CCyR rate [52.6% (10/19) vs 0 (0/9) ] were higher among patients treated with LEN (n=29) than those treated with non-LEN therapy (n=25) . There was no statistically significant difference in OS between the patients with LEN or non-LEN therapy (78 months vs 62 months, P=0.297) . Conclusion Comparing del (5q) syndrome patients with isolated del (5q) or with del (5q) + ACA, two groups of patients had similar clinical characteristics, median OS and LEN efficacy. LEN showed better treatment response than traditional drugs in patients with del (5q) syndrome.

OBJECTIVETo analyze the clinical, laboratory characteristics and PIG-A gene mutations in patients of myelodysplastic syndromes (MDS) with PNH clones.

METHODS218 MDS patients diagnosed from August 2013 to August 2015 were analyzed. The PIG-A gene mutations were tested in 13 cases of MDS with PNH clones, 17 cases of AA-PNH and 14 cases of PNH selected contemporaneously by PCR and direct sequencing.

RESULTS13 (5.96%) MDS patients were detected with PNH clones (13/218 cases). 9 patients were treated with cyclosporin A (CsA). Patients showed hematological improvement (HI). There were significant differences between MDS-PNH and PNH patients in terms of granulocyte clone size, red cell clone size and LDH levels [19.2% (1.0%-97.7%) vs 60.2% (3.1%-98.0%), P=0.007; 4.3% (0-67.2%) vs 27.9% (2.5%-83.6%), P=0.026; 246 (89-2014) U/L vs 1137 (195-2239) U/L, P=0.049], while the differences were not statistically significant in patients between MDS-PNH and AA-PNH patients [19.2% (1.0%-97.7%) vs 23.2% (1.5%-96.0%), P=0.843; 4.3% (0-67.2%) vs 14.4% (1.1%-62.8%), P=0.079; 246 (89-2014) U/L vs 406 (192-1148) U/L, P=0.107]. PIG-A gene mutations were detected in 7 MDS-PNH patients, of them, six were missense mutations, one were frameshift mutation and four cases with the same mutation of c.356G>A (R119Q). The PIG-A gene mutations were also detected in 9/11 AA-PNH patients and 11/14 PNH patients, both of them had the mutation of c.356G>A (R119Q). The PIG-A gene mutations of MDS-PNH, AA-PNH, PNH patients were all small mutations, the majority of those (59%) were missense mutation and mainly located in exon 2.

CONCLUSIONMDS patients with PNH clones had better response to CsA, smaller PNH clone size. The PIG-A gene mutations of MDS-PNH patients mainly located in exon 2, which could be a mutational hotspot of these patients.

Anemia, Aplastic ; genetics ; Clone Cells ; Erythrocytes ; cytology ; Exons ; Granulocytes ; cytology ; Hemoglobinuria, Paroxysmal ; genetics ; Humans ; Membrane Proteins ; genetics ; Mutation ; Myelodysplastic Syndromes ; genetics ; Polymerase Chain Reaction

OBJECTIVETo estimate the long-term outcomes and the prognostic factors of homoharringtonine, cytarabine, daunorubicin or idarubicin (HAD/HAI) as induction chemotherapy in de novo acute myeloid leukemia (AML).

METHODSThe CR rate, overall survival (OS) rate, relapse free survival (RFS) rate were retrospectively assayed in 143 de novo AML patients who received the HAD/HAI induction chemotherapy. The outcomes were compared among prognostic groups according to world health organization (WHO) classification, genetic prognosis and initial white blood cell (WBC) count. The role of consolidation chemotherapy consisting of middle-dosage Ara-C (MD-Ara-C) on long term survival was evaluated.

RESULTSOf 143 patients, 112 (78.3%) achieved CR after the first course of HAD/HAI induction treatment, and early death occurred in only one case. Notably, the CR rate of patients with an initial WBC count ≥100×10(9)/L was not significantly different from those with an initial WBC count<100× 10(9)/L (70.4% vs 80.2%, P=0.266). The CR rate for the patients with favorable, intermediate and unfavorable integrated genetics risk factors was 93.7%, 71.4% and 61.3%, respectively, the difference between groups was statistically significant (P=0.001). Patients with FLT3-ITD mutation obtained similar CR rate (70.6%) to that of patients with FLT3 wild type (79.3%, P=0.528).The estimated 5-year OS rate and 5-year RFS rate for all patients was 40.0% and 37.0%, respectively, with a median follow-up of 24 (range 1-104) months. The median survival time was 30 [95%CI (12, 48)] months. 5-year OS and 5-year RFS of the 96 patients who achieved CR after first course chemotherapy without undergoing allo-HSCT in complete remission was 47.0% and 38.0%, respectively. 5-year OS was significantly higher in MD-Ara-C consolidation group than in no MD-Ara-C consolidation group among CR patients without allo-HSCT (58.0%, 19.0%, respectively, P=0.004). In patients who obtained CR after first course and received MD-Ara-C consolidation without allo-HSCT, the 5-year OS of patients with hyperleukocytosis was not significantly lower than that of patients without hyperleukocytosis (55.5%, 58.8%, respectively,P=0.419). FLT3-ITD mutation patients showed similar 5-year OS to that of wild type FLT3 patients (51.4%, 60.2%, respectively, P=0.482). And furthermore, 5-year OS of favorable, intermediate and unfavorable integrated genetics groups were 59.1%, 62.5%, 51.9%, respectively (P=0.332) in this subgroup.

CONCLUSIONHAD/HAI induction chemotherapy with sequential consolidation of MD-Ara-C could obtain satisfactory CR rate and long-term survival rate in de novo AML, especially for patients with hyperleukocytosis or FLT3-ITD mutation. It yet remains to be verified by large sample, prospective studies.

Cytarabine ; therapeutic use ; Daunorubicin ; therapeutic use ; Harringtonines ; therapeutic use ; Humans ; Idarubicin ; therapeutic use ; Induction Chemotherapy ; Leukemia, Myeloid, Acute ; drug therapy ; Leukocyte Count ; Prognosis ; Prospective Studies ; Remission Induction ; Retrospective Studies ; Survival Rate

OBJECTIVETo investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.

METHODSA cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.

RESULTSFatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).

CONCLUSIONMPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.

Humans ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; diagnosis ; physiopathology ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; complications ; Thrombocythemia, Essential ; complications

Objective: To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees. Methods: Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated. Results: 320 subjects (47%) presented severe thrombocytopenia (PLT<50×10⁹/L), 198 ones (15.2%) mild thrombocytopenia [PLT (50-99)×10⁹/L] and 787 ones (60.3%) without thrombocytopenia (PLT ≥ 100×10⁹/L). The more severe the thrombocytopenia, the higher the proportions of HGB<100 g/L, WBC<4×10⁹/L, circulating blasts ≥ 3%, abnormal karyotype and unfavourable cytogenetics (P<0.001, P<0.001, P=0.004, P<0.001 and P<0.001, respectively) were observed in this cohort of patients. The more severe the thrombocytopenia, the lower the proportion of JAK2V617F positive (P<0.001) was also noticed. Platelet count was positively correlated with splenomegaly, HGB and WBC (P<0.001, correlation coefficients were 0.131, 0.445 and 0.156, respectively). Platelet count was negative correlated with constitutional symptoms and circulating blasts (P=0.009, P=0.045, respectively; correlation coefficients were -0.096 and -0.056, respectively). The median survival of patients with severe thrombocytopenia, mild thrombocytopenia and without thrombocytopenia were 32, 67 and 89 months, respectively (P<0.001). Multivariate analysis identified thrombocytopenia in varied degrees (HR=1.693, 95%CI 1.320-2.173, P<0.001) and Dynamic Internation Prognostic Scoring System(DIPSS) prognostic model (HR=2.051, 95%CI 1.511-2.784, P<0.001) as independent risk factors for survival. Conclusion PMF patients with severe thrombocytopenia frequently displayed anemia, leucopenia, circulating blasts and short survival, so active treatment measures should be taken especially in these patients.