Objective To explore the efficacy and adverse reactions of improved VTD regimen (pirarubicin combined with vincristine and dexamethasone) plus low-dose thalidomide in patients of newly diagnosed multiple myeloma(MM).Methods Twenty-nine cases of newly diagnosed MM were enrolled in this study.The improved VTD regimen was intravenous injection vincristine 2 mg/d on the first day,intravenous drip pirarubicin 20-30 mg/d from the first day to the second day,and intravenous drip dexamethasone 8 mg/d from the first day to the tenth day.Twenty-eight days was one course of treatment.Response and adverse reactions were evaluated after 4 course of treatment.On the first day of chemotherapy,all the patients were orally administered thalidomide 50 mg/d.Three days later,thalidomide was added to 100 mg/d and chronically maintained if toxicities could be tolerated.Results There were 3 cases(10.3％) in complete response,12 cases (41.2％) in very good partial response,10 cases (34.5％) in partial response,3 cases (10.3％) in stable disease,and 1 case(3.5％) in progressive disease.The overall response rate was 86.2％.Main adverse reactions were myelosuppression,asthenia and constipation,all could be tolerated.Conclusion It has significant response rate and less side effects of improved VTD regimen plus low-dose thalidomide for the patients of newly diagnosed multiple myeloma,and deserves further clinical practice.

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Objective:To investigate the changes of related indicators of right heart hypofunction in patients with primary myelofibrosis (PMF).Methods:The clinical data of 55 PMF patients in the Second People's Hospital of Lianyungang in Jiangsu Province and Jiangsu Province Hospital from January 2015 to August 2019 were retrospectively analyzed. The differences in right heart function-related echocardiographic indexes and biochemical indexes between pre-fibrosis/early stage fibrosis patients and obvious stage fibrosis patients were compared. Single factor linear regression method was used to analyze the correlations of pulmonary artery pressure with biochemical indexes.Results:The hemoglobin level [119 g/L (47-224 g/L) vs. 78 g/L (33-182 g/L)] and platelet count [233×10 12/L (5×10 12/L-984×10 12/L) vs. 117×10 12/L (7×10 12/L-731×10 12/L)] of patients in the pre-fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group, and the differences were statistically significant (both P<0.05). Among 22 patients with complete results of cardiac ultrasound, 90.9% (20/22) patients had increased pulmonary artery pressure, 72.7% (16/22) patients had increased left atrial diameter, and 90.9% (20/22) patients had increased right ventricular diastolic diameter. There were no patients with abnormal ejection fraction. The pulmonary artery pressure [48 mmHg (46-90 mmHg) vs. 33 mmHg (20-50 mmHg) (1 mmHg = 0.133 kPa)], left ventricular diastolic diameter [46 mm (36-50 mm) vs. 47 mm (43-53 mm)] and fractional shortening rate [38.1% (36.0%-38.9%) vs. 35.4% (32.7%-37.8%)] of patients in the pre-fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group, and the differences were statistically significant (all P < 0.05). The pulmonary artery pressure of patients had positive correlations with age ( r = 0.590), serum ferritin (SF) ( r = 0.608), lactate dehydrogenase (LDH) ( r = 0.711) and soluble growth-stimulating expression gene 2 (ST-2) ( r = 0.580)(all P<0.05), and had negative correlation with platelet count ( r = -0.596, P = 0.003). Conclusion:PMF patients are prone to right heart hypofunction, the pulmonary artery pressure is higher in older patients and patients with high SF, LDH and ST-2 levels and low platelet count.

Objective To investigate the MRI manifestations of pilomyxoid astrocytoma (PMA)at unusual sites and differential diagnosis in children.Methods The clinical and MRI data from 6 cases of PMA with atypical locations confirmed by surgery and pathology were analyzed retrospectively.Results 2 cases located at cerebral hemisphere,1 case at tegmentum mesencephali,1 case at medulla and 2 cases at cerebellar hemisphere.There were 4 cases of solid tumor and 2 case belonged to cystic-solid mass.5 cases were round or oval and 1 case was shallow lobulated.In addition,there were 2 cases with hemorrhage and 2 cases with peritumoral edema.T1WI showed hypointensity,isointense or slightly hyperintense while T2WI and fluid attented inversion recovery (FLAIR)exhibited isointensity or hyperintensity.After contrast administration,there were 3 cases of obvious multi-ring enhancement,of which 1 case showed astral enhancement of the mural nodules and the other 3 cases showed mild homogeneous or inhomogeneous enhancement.4 cases showed non-enhanced areas in the solid parts.No signs of leptomeningeal dissemination were found in all 6 cases.Conclusion The PMA at unuaual sites in children is primarily solid or cystic-solid,and the image manifestations of plain scan are varied.After enhancement,the tumors show obviously multi-circular enhancement and mild homogeneous or inhomogeneous enhancement could be observed,no enhancement areas are visible in the solid parts.It is noteworthy to distinguish this astrocytoma from other lesions.

Objective To analyze some related causes of hypocytosis or hematocytosis with bone marrow dry pumping. Methods Bone marrow histology, reticular fiber staining and selective immunohistochemical staining were performed in 34 bone marrow dry pumping patients with hypocytosis or hematocytosis in the Second People's Hospital of Lianyungang from January 2012 to August 2017. Results All the patients showed dizziness, fatigue, splenomegaly, night sweats and bleeding, including primary myelofibrosis (17 cases, 50.0 %), chronic myelocytic leukemia (4 cases, 11.8 %), acute myelocytic leukemia (2 cases, 5.9 %), acute lymphoblastic leukemia (1 case, 2.9 %), myelodysplastic syndrome (2 cases, 5.9 %), multiple myeloma (2 cases, 5.9 %), non-Hodgkin lymphoma with bone marrow infiltration (2 cases, 5.9 %), polycythemia vera (1 case, 2.9 %) and bone marrow metastatic tumor (3 cases, 8.8 %). The bone marrow proliferative degree in primary myelofibrosis group was mainly "grade Ⅱ" to "grade Ⅳ", and the non primary myelofibrosis group was mainly "grade Ⅲ" to "grade Ⅴ", and the differences of proliferative degree component between them were statically significant (χ2＝ 12.900, P＝ 0.004). The fibrosis level in primary myelofibrosis group was mainly "grade 2" to "grade 3", and the non primary myelofibrosis group was mainly "grade 1" to "grade 2", and the differences of myelofibrosis degree component between them were also statistically significant (χ2＝ 12.692, P＝ 0.003). Conclusions Hematological malignancies, especially primary myelofibrosis, are the common causes of bone marrow "dry pumping". Bone marrow histology, reticular fiber staining and selective immunohistochemical staining are of great significance in the etiological diagnosis.

Objective To investigate the clinical and laboratory features of IgG-2κ light chain multiple myeloma. Methods The clinical data and laboratory results of 2 multiple myeloma (MM) patients with IgG-2κ light chain were analyzed and the related literatures were reviewed. Results Two male and 1 female patients were 50-82 years old and mainly suffered with backache, infection, anemia and renal dysfunction. Multiple osteolytic bone destruction was detected in X-ray as well as magnetic resonance imaging (MRI). The level of serum IgG was normal, slight or obviously increased, but the levels of IgA and IgM were decreased. The levels of κ light chain in serum and urine were both increased significantly, and Bence-Jones protein was positive. Double M protein peaks of serum in γ area were detected by protein electrophoresis in 2 patients. A single band of IgG and double bands of light chain κ were revealed by immunofixation electrophoresis. Bone marrow smear showed that abnormal plasma cells were increased obviously. One patient gave up chemotherapy because of lung infection, acute left heart failure and acute renal failure, the others 2 patients achieved partial remission and stable disease by receiving DVD and VAD chemotherapy. Conclusions IgG-2κ light chain MM lacks typical clinical presentation, but some laboratory characteristics may be different from those of IgG-κ light chain. Further researches are needed to confirm whether or not it belongs to biclonal MM.

Objective:To improve the cognition of T-cell large granular lymphocytic leukemia (T-LGLL) combined with pure red cell aplasia (PRCA).Methods:The clinical characteristics, peripheral blood and bone marrow laboratory indicators of 14 newly diagnosed patients with T-LGLL combined with PRCA who were admitted to the Second People's Hospital of Lianyungang Affiliated to Bengbu Medical College and the People's Hospital of Jiangsu Province from August 2010 to October 2019 were retrospectively analyzed.Results:Among the 14 patients, there were 7 males and 7 females, with a median age of 58.5 years (33-75 years). At the first visit, the median white blood cell count was 5.02×10 9/L [(1.45-8.49)×10 9/L], the median absolute value of neutrophils was 1.35×10 9/L [(0.43-7.16)×10 9/L], the median lymphocyte ratio was 0.49 (0.13-0.77), the median hemoglobin was 58 g/L (42-106 g/L), the median red blood cell count was 2.01×10 12/L [(0.99-3.20)×10 12/L], the median reticulocyte count percentage was 0.52 (0.14-3.02), the median platelet was 96×10 9/L [(38-281)×10 9/L], the median large granular lymphocytes accounted for 71% (32%-81%) of lymphocytes. Bone marrow aspiration showed that the median large granular lymphocytes accounted for 0.16 (0.08-0.41) of nuclear cells, and the median serum β 2 microglobulin was 4.85 mg/L (2.81-7.22 mg/L). Two patients had ASXL1 and TET2 mutations, and one of them had STAT3, EP300 and FAM46C mutations. Six patients were T cell receptor (TCR) β and γ-positive, 1 patient were TCRβ-positive, 4 patients were TCRγ-positive, 1 patient was TCRδ-positive, 1 patient was TCRβ, γ and δ-positive, and 1 patient was all negative. Eight cases received cyclosporine therapy, 6 cases were effective; 6 cases received methotrexate combined with hormone therapy, 3 cases were effective. The initial induction therapy was effective in 9 cases, 5 patients who failed in the initial treatment received salvage treatment, and 2 cases were effective. Conclusions:The laboratory characteristics of patients with T-LGLL combined with PRCA are similar to those of simple T-LGLL, anemia is a prominent manifestation accompanied by neutropenia or thrombocytopenia. The large granular lymphocytes are easily seen in peripheral blood and bone marrow, and T monoclonal rearrangement of lymphocytes is an important feature, and the patients respond well to immunosuppressive therapy.

Objective:To investigate the effect of CUEDC1 gene on the acute monocytic leukemia THP-1 cells gene expression profile.Methods:The differential expression gene bank of THP-1 cells with CUEDC1 gene interference was constructed. The differential gene expression of THP-1 cells in CUEDC1 interference group and the negative control group was compared based on RNA sequencing technology. The part of the differentially expressed genes were verified by using real-time polymerase chain reaction (PCR), and the up-regulated and down-regulated genes were respectively imported into DAVID software for gene ontology (GO) function enrichment analysis.Results:The differentially expressed gene bank of THP-1 cells interfered by CUEDC1 gene was successfully constructed. A total of 161 differentially expressed genes were detected in CUEDC1 interference group and the negative control group ( P < 0.05), including 85 up-regulated genes and 76 down-regulated genes. There were 9 genes related to cell proliferation and 10 genes related to apoptosis, 2 genes related to p53 gene and 3 genes related to transcriptional regulation, 8 genes related to ubiquitin, among which SMAD5, SG15, CBLL1, FANCF and other genes were closely related to the occurrence and development of acute leukemia. Conclusion:CUEDC1 gene participates in the occurrence and development of acute monocytic leukemia by influencing the expressions of SMAD5, SG15, CBLL1, FANCF and other genes.

Objective:To explore the ultrasonography and classification management strategy for adult atrial septal aneurysm (ASA).Methods:Of 138 patients diagnosed with ASA by the transthoracic echocardiography (TTE), in No.83 Army Hospital and No.988 Hospital of Joint Logistic Support Force from January 2015 to October 2019, 103 underwent the transesophageal echocardiography (TEE), and all were examined by the right heart contrast transthoracic echocardiography (cTTE). According to the semi-quantitative method, the shunt was graded, classified, and managed through follow-up or surgical treatment.Results:In the resting state, among 138 patients, 26 cases were detected by TTE as ASA with patent foramen ovale (PFO), showing left-to-right shunt. Among 112 patients with no shunt detected by TTE, 103 cases underwent TEE, of whom 44 cases had PFO and left-to-right shunt. The detection rate of PFO shunt by TEE was significantly higher than that of TTE (42.72% vs 18.84%, P<0.001). Out of the 138 patients who underwent cTTE, 96 cases had different degrees of right-to-left shunt. According to the semi-quantitative standard, there were 15 cases of grade Ⅰ, 28 cases of grade Ⅱ, and 53 cases of grade Ⅲ. The detection rate of cTTE was significantly higher than that of TTE and TEE (69.56% vs 18.84% and 42.72%, both P<0.001). Among these 96 patients with right-to-left shunt detected by cTTE, 52 cases (54.17%) were clinically diagnosed as cryptogenic stroke, with right-to-left shunt in 6 cases of grade Ⅰ, 12 cases of grade Ⅱ, and 34 cases of grade Ⅲ. The number of new infarcts in patients with grade Ⅱ or Ⅲ shunts who received PFO closure via catheter was significantly lower than that of patients who did not receive closure therapy (0 vs 27.27%, P=0.011). Conclusion:ASA combined with PFO and right-to-left shunts were detected more frequently by ultrasonography combined with cTTE in adults, this helps to formulate a reasonable management strategy.