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Efficacy and safety of high-dose dexamethasone combined with rhTPO for newly diagnosed adults with severe immune thrombocytopenia.

Yan LI ; Qin HUANG ; Chao WANG ; Muhebaier ; Li AN ; Xiaomin WANG

Chinese Journal of Hematology.2016;37(2):134-137.

OBJECTIVETo evaluate the efficacy and safety of high dose dexamethasone combined with recombinant human thrombopoietin (rhTPO) in adults with severe newly diagnosed immune thrombocytopenia (ITP).

METHODSForty-eight adult patients with severe ITP were randomized into two groups, experimental group and control group. The patients in experimental group were given high-dose dexamethasone combined with rhTPO treatment, the patients in control group were given single high-dose dexamethasone treatment. Platelet count, platelet increase, as well as the overall response rate were strictly observed in the process. At the same time, the patient's drug tolerance and any adverse drug reactions were observed.

RESULTSThe platelet counts and platelet increase of the patients in experimental group were significantly higher than that in control group (P<0.05) at day 3, 7, 14, 30. There was no significant difference in overall response rates between the two groups (34.8% vs 36.0%, 56.5% vs 48.0%, P>0.05) at day 3, 7. The overall response rates of experimental group at day 14, 30 were significantly higher than that of control group (91.3% vs 68.0%, 82.6% vs 52.0%, P<0.05). The muscle aches occurred in one patient in experimental group which was self-recovery without special treatment.

CONCLUSIONrhTPO combined with high-dose dexamethasone could rapidly increase the platelet count, reduce the risk of bleeding, and prolonge the effect with a low incidence of tolerable adverse events compared to single high-dose dexamethasone. rhTPO combined with high-dose dexamethasone could be a new therapeutic choice for severe primary ITP.

Adult ; Blood Platelets ; Dexamethasone ; administration & dosage ; therapeutic use ; Humans ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; drug therapy ; Recombinant Proteins ; administration & dosage ; therapeutic use ; Thrombopoietin ; administration & dosage ; therapeutic use ; Treatment Outcome

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Study on the targeting effects of M1-GS RNA on K562 cells.

Bo-bin CHEN ; Guo-wei LIN ; Hua-zhong LU ; Hua-hua FAN ; Li GAO

Chinese Journal of Hematology.2004;25(9):552-555.

OBJECTIVETo determine the effects of M1-GS RNA (M1 RNA) on bcr-abl mRNA and oncoprotein after M1 RNA with guide sequence (M1-GS RNA) targeting the oncogene was transfected into K562 cells.

METHODSpAVGS4 (an eukaryocyte expression vector containing M1-GS RNA sequence) and pNAV-1 (as the control) were transfected into K562 cells by X-tremeGENE Q2. Total RNA was extracted at 24, 48, 72 and 96 hours after transfection. Then RT-PCR was done to compare the products at different time point. After collecting pAVGS4-transfected cells and the control cells at 48 and 96 hours after transfection, total protein was extracted and quantified. Change of P210 was determined by Western blot. Colony formation was analyzed at 96 hours after transfection.

RESULTSRT-PCR based on transfected cells at different time point showed that the amount of bcr-abl mRNA began to decrease at 24 hours and reduced to 9.2% and 2.5% respectively at 48 and 72 hours after transfection. Western blot showed that the expression of P210 in the pAVGS4 group reduced to 10.4% of the control at 48 hours and 6.7% of the control at 96 hours after transfection. The inhibition rate of colony formation was 81.3% after K562 cells were transfected by pAVGS4.

CONCLUSIONpAVGS4 can efficiently destroy bcr-abl mRNA in K562 cells. The transcript level of bcr-abl mRNA was reduced with the time after transfection. The expression of P210 was decreased significantly at 48 and 96 hours after transfection. K562 cell colony formation was prominently inhibited.

Escherichia coli Proteins ; genetics ; Fusion Proteins, bcr-abl ; genetics ; metabolism ; Genetic Vectors ; Humans ; K562 Cells ; RNA, Bacterial ; genetics ; RNA, Catalytic ; genetics ; RNA, Messenger ; genetics ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Ribonuclease P ; genetics ; Time Factors ; Transfection ; methods

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Clinical and molecular-biological study of a May-Hegglin anomaly family.

Xiu-ru SHAO ; Jia-zeng LI ; Jun MA ; Zhao-min ZHAN ; Hong LIANG ; Xi-nan SHE ; Hai-ling LU ; Lai-ci WANG ; Chui-ming JIA ; Li-jie WU ; Ming-hua JIN ; Li-jun CHEN

Chinese Journal of Hematology.2004;25(9):548-551.

OBJECTIVETo study the changes of platelet in May-Hegglin anomaly (MHA) and the molecular pathogenesis mechanism.

METHODSPeripheral blood was drawn from the MHA proband, her father and her uncle. Platelet count and morphology were examined by automatic blood cell counter and microscopy, respectively. The platelet membrane protein was examined by flow cytometry. Membrane antibodies were determined by ELISA. PCR was used to amplify the exons 25, 31 approximately 32, 38 and 40 of the MYH 9 gene in the MHA patient and her diseased father. Furthermore, PCR products were sequenced, a specific point mutation was identified and inclusions (Dohle's body) in the neutrophil was detected by indirect immunofluorescence technique.

RESULTSIt was proved that in MHA patients, platelet count was higher by cell counter than by microscope (P < 0.01). Giant platelet was 94% but platelet membrane proteins (CD41, CD61, CD42A, CD42b) were in normal range. Membrane antibodies was undetectable. An A5521G mutation (GAG-->AAG) in the exon 38 was found in the proband and her diseased father, resulting in a characteristic change of NMMHC-A1841 (Glutamic acid-->Arginine), which was not found in other members of the family and in normal controls. Spindle-like inclusions with fluorescence were clearly displayed in neutrophil cytoplasm.

CONCLUSIONThe molecular pathogenesis mechanism of May-Hegglin anomaly is the mutation in MYH 9 gene.

Adult ; Base Sequence ; Blood Platelets ; metabolism ; pathology ; DNA Mutational Analysis ; Enzyme-Linked Immunosorbent Assay ; Female ; Flow Cytometry ; Granulocytes ; metabolism ; pathology ; Humans ; Inclusion Bodies ; metabolism ; pathology ; Male ; Molecular Motor Proteins ; genetics ; Mutation ; Myosin Heavy Chains ; genetics ; Pedigree ; Platelet Count ; Platelet Membrane Glycoproteins ; metabolism ; Thrombocytopenia ; blood ; genetics ; pathology

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Effects of 2A-1-1 on the aggregation and Ca2+ influx of platelets.

Fu-ren ZENG ; Song-mei YIN ; Shuang-feng XIE ; Da-nian NIE ; Li-ping MA ; Jian-hong FENG ; Li-zhuo XU ; Yong-yuan GUAN

Chinese Journal of Hematology.2004;25(9):544-547.

OBJECTIVETo explore the effects of 2A-1-1 (purified component from Panax notoginsengs saponins) on the aggregation of and Ca2+ influx into human platelets.

METHODSThe aggregation of platelets was tested by nephelometry, Fura-2 fluorescent technique was used for detecting cell [Ca2+]i. The effects of 2A-1-1, nifedipine and SK&F96365 on Ca(2+) influx into human platelets induced by ADP or CPA were observed separately.

RESULTSNifedipine (< 20 micromol/L) could not inhibit platelet aggregation induced by ADP or the Ca(2+) influx induced by ADP or CPA. SK&F96365 at 20 micromol/L could inhibit the maximal aggregation of platelets induced by ADP with a inhibitory rate of 59.83%, at 15 micromol/L could inhibit the Ca2+ influx induced by CPA or ADP. 2A-1-1 (5, 10 and 20 micromol/L) could inhibit the maximal aggregation of platelets induced by ADP with the inhibitory rates of 47.06%, 53.47% and 71.52%, respectively. 2A-1-1 at 10 and 20 micromol/L could inhibit the Ca2+ influx induced by CPA or ADP.

CONCLUSIONS2A-1-1 can inhibit platelets aggregation, block the ROC (Receptor-dependent Ca2+ channels) and inhibit Ca2+ influx of human platelets.

Adenosine Diphosphate ; pharmacology ; Adult ; Blood Platelets ; cytology ; drug effects ; metabolism ; Calcium ; metabolism ; pharmacokinetics ; Calcium Channel Blockers ; pharmacology ; Dose-Response Relationship, Drug ; Female ; Ginsenosides ; pharmacology ; Humans ; Imidazoles ; pharmacology ; Indoles ; pharmacology ; Male ; Nifedipine ; pharmacology ; Platelet Aggregation ; drug effects ; Platelet Aggregation Inhibitors ; pharmacology

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A study of human annexin V derivative: its effects of anticoagulation and antithrombosis.

Cheng-wei JU ; Lian-sheng WANG ; Xiang YANG ; Gen-shan MA ; Zi-chun HUA ; Xing-ya GAO

Chinese Journal of Hematology.2004;25(9):540-543.

OBJECTIVETo investigate the effects of a new anticoagulant, annexin V derivative (AND) on anticoagulation and antithrombosis.

METHODSHigh and low doses of AND were given to rabbits (groups 1 and 2 respectively) by intravenous (iv) bolus injections followed by half the respective AND doses by iv infusion over 2 hours. Control groups were iv given heparin (group 3) and saline (group 4) of the same volume and procedure as that in group 1 and 2. Blood cell count, activated partial thromboplastin time (APTT), prothrombin time (PT), thrombin time (TT) and fibrinogen level were examined before and 15, 30 and 60 min after iv bolus and 2 hours after the end of iv infusion. A 3.0 mm x 15 mm balloon was put into femoral artery to induce endothelial denudation 15 min after IV bolus and the blood pressure of femoral artery was monitored until the pulse pressure recorded 0 mm Hg when the vessel was occluded completely by a thrombus. The femoral arteries were collected and the thrombi were stripped off for measuring their lengths, wet and dry weights.

RESULTSAnticoagulation parameters: APTT at 15 min after iv bolus in AND group was significantly longer than that in group 4 (P < 0.05) but shorter than that in group 3 (P < 0.05); APTT and TT in group 3 were significantly longer than those in groups 1, 2 and 4. Fibrinogen: 0.70 mg/kg AND may decrease fibrinogen. Antithrombosis values: the wet and dry weights in AND groups were significantly lighter than those in group 3 and 4 (P < 0.05). The dry weight in high-dose AND group was remarkably lighter than that in low-dose group (P = 0.029). The length of thrombus in low-dose AND group was remarkably shorter than that in group 4 (P = 0.013), but not for group 3 (P > 0.05). It was remarkably shorter in high-dose AND group than in both group 3 (P < 0.001) and 4 (P = 0.015). The time when pulse pressure equaled to 0 was longer in AND group than in group 4 (P < 0.05), but not in 3.

CONCLUSIONAND is an effective anticoagulant and antithrombosis agent, the highest anticoagulation effect occurs at 15 min after IV bolus. Its anticoagulation effect is not more potent than that of standard heparin, while antithrombosis capacity is more effective. AND in treating thrombosis clinically might be promising.

Animals ; Annexin A5 ; administration & dosage ; pharmacology ; Anticoagulants ; administration & dosage ; pharmacology ; Blood Coagulation ; drug effects ; Disease Models, Animal ; Fibrinogen ; analysis ; Humans ; Injections, Intravenous ; Male ; Partial Thromboplastin Time ; Prothrombin Time ; Rabbits ; Random Allocation ; Thrombin Time ; Thrombosis ; prevention & control

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Hereditary hemorrhagic telangiectasia resulted from a nonsense mutation Arg479 Stop in the ALK-1 gene.

Bing-shou XIE ; Shuang XIE ; Ping CHEN ; Miao-yong ZHU ; Jia-yong ZHENG ; Xue-feng WANG ; Qi-hua FU ; Rong-fu ZHOU ; Wen-bin WANG ; Wen-man WU ; Qiu-lan DING ; Hong-li WANG ; Li-ming HU

Chinese Journal of Hematology.2004;25(9):536-539.

OBJECTIVETo identify the gene mutations in a pedigree with hereditary hemorrhagic telangiectasia.

METHODSGenomic DNA was extracted from the peripheral blood of the propositus. All of the exons, intron/exon boundaries and the 5' untranslation regions (UTR) of the ALK-1 and endoglin gene were amplified by polymerase chain reaction (PCR). The PCR products were screened by direct sequencing.

RESULTSThe mutation is a C1437T substitution in exon 10 of the ALK-1 gene, resulting in Arg 479 Stop.

CONCLUSIONThe hereditary hemorrhagic telangiectasia propositus is caused by a heterozygous Arg 479 Stop mutation in the ALK-1 gene which has not been identified previously.

Activin Receptors, Type II ; genetics ; Aged ; Antigens, CD ; genetics ; Base Sequence ; Codon, Nonsense ; DNA Mutational Analysis ; Exons ; genetics ; Female ; Humans ; Male ; Pedigree ; Point Mutation ; Receptors, Cell Surface ; genetics ; Telangiectasia, Hereditary Hemorrhagic ; genetics ; pathology

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Effects of vascular endothelial growth factor on differentiation and function of dendritic cells generated from CD34+ hematopoietic progenitor cells in vitro.

Feng YE ; Huai-zeng CHEN ; Xing XIE ; Da-feng YE

Chinese Journal of Hematology.2004;25(9):532-535.

OBJECTIVETo investigate the effects of vascular endothelial growth factor (VEGF) on differentiation and function of dendritic cells derived from CD34+ hematopoietic progenitor cells.

METHODSAfter isolation from umbilical cord blood with a high-gradient magnetic cell sorting system (MACS), the CD34+ cells were cultured with a cocktail cytokines for differentiating into dendritic cells (DC). The cells were stimulated by VEGF (25 ng/ml) either at the beginning or at day 9 of culture. Kinetics analysis of cell proliferation was performed during the process of cell culture, and the expression of DC differentiation antigens including CD1alpha, CD83, CD80, CD54 and HLA-DR was examined by flow cytometry. DC function was evaluated by the ability to induce proliferation of allogeneic T cells in mixed lymphocyte reaction (MLR) assay, and the production of IL-12 by ELISA.

RESULTSVEGF added at day 1 of culture induced an increase of total cell numbers by (1.51 +/- 0.23)-folds (P = 0.001). VEGF added at the initial but not the late stage of culture could dramatically down-regulate the expression of CD1a [(33.00 +/- 2.12)% vs (81.20 +/- 6.93)%], CD83 [(42.23 +/- 1.15)% vs (87.98 +/- 7.97)%], CD80 (42.93 +/- 1.32)% vs (94.53 +/- 0.87)%], and HLA-DR [(37.93 +/- 5.30)% vs (74.15 +/- 3.74)%], while obviously up-regulate the expression of CD14. Moreover, the inhibitory effect of VEGF on DC function was confirmed by a reduced ability to induce proliferation of allogeneic T cells and production of IL-12 (P < 0.01).

CONCLUSIONSVEGF could induce the expansion of hematopoietic progenitor cells and inhibit at the early stage their differentiation into mature DC.

Antigens, CD ; analysis ; Antigens, CD1 ; analysis ; Antigens, CD34 ; analysis ; blood ; B7-1 Antigen ; analysis ; Cell Differentiation ; drug effects ; Cell Proliferation ; drug effects ; Cells, Cultured ; Dendritic Cells ; cytology ; immunology ; metabolism ; Enzyme-Linked Immunosorbent Assay ; Fetal Blood ; cytology ; immunology ; Flow Cytometry ; HLA-DR Antigens ; analysis ; Hematopoietic Stem Cells ; cytology ; immunology ; metabolism ; Humans ; Immunoglobulins ; analysis ; Intercellular Adhesion Molecule-1 ; analysis ; Interleukin-12 ; analysis ; Membrane Glycoproteins ; analysis ; Vascular Endothelial Growth Factor A ; pharmacology

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Report of 8 cases of bcr-abl gene positive thrombocytosis and review of the literature.

Feng-ru LIN ; Yan WANG ; Jing CHEN

Chinese Journal of Hematology.2004;25(9):528-531.

OBJECTIVETo analyse the features of 8 cases of Bcr(+) thrombocytosis.

METHODSThe clinical and hematological features and therapeutic outcomes were studied retrospectively in 8 Bcr(+) thrombocytosis and compared with essential thrombocytosis (ET) and chronic myeloid leukemia-chronic phase thrombocytosis (CML-CP-T). BCR-ABL fusion gene was detected with PCR.

RESULTS(1) Except for the presence of BCR-ABL fusion gene, there was no significant difference in clinical and hematological features and therapeutic outcomes between thrombocytosis with or without BCR-ABL. (2) The Bcr(+) thrombocytosis differed from CML-CP-T in the following aspects: female predominance, milder or no splenomegaly, peripheral leukocytes count < 40 x 10(9)/L, less or no basophilia and fewer immature granulocytes in peripheral blood, bone marrow granulocytic and/or megakaryocytic lineage hyperplasia, normal or increased neutrophil alkaline phosphatase score and less blastic transformation.

CONCLUSIONBcr(+) thrombocytosis may be considered as a new member of chronic myeloproliferative diseases, a variant of essential thrombocythemia.

Adult ; Aged ; Female ; Fusion Proteins, bcr-abl ; genetics ; Humans ; Leukemia, Myeloid, Chronic-Phase ; genetics ; pathology ; therapy ; Male ; Middle Aged ; Prognosis ; Thrombocytosis ; genetics ; pathology ; therapy ; Young Adult

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The effects of tissue factor/activated factor VII complex on the invasion and metastasis of human ovarian cancer.

Jun FANG ; Wen-ning WEI ; Ling-hui XIA ; Shan-jun SONG

Chinese Journal of Hematology.2004;25(9):523-527.

OBJECTIVETo explore the role of tissue factor/activated factor VII (TF/FVIIa) complex in human ovarian cancer invasion and metastasis.

METHODS(1) Constructed an expression vector of TF, pcDNA3-TF and established a human ovarian cell line A2780/TF expressing high level TF by using molecular cloning and gene transfection techniques. (2) By Boyden chamber assay to count the numbers of A2780 and A2780/TF cells that penetrated the matrigel to the back of PVPF membrane after FVIIa stimulation. (3) BALB/c nude mice were used to establish experimental model of metastasis with A2780 or A2780/TF and the lung tissue sections were examined by microscopy for cancer metastasis.

RESULTS(1) Compared with their parental A2780 cells, A2780/TF cells expressed high level of TF mRNA (3.99 +/- 0.15 vs 0.97 +/- 0.23, P < 0.01) and TF antigen on cell surface \[(48.56 +/- 9.53)% vs (2.73 +/- 1.15)%, P < 0.01\]. (2) After stimulation, the A2780/TF cell number on the back of PVPF membrane increased from basal level 157.3 +/- 19.2 to 447.7 +/- 39.4 (P < 0.01), which could decreased to basal level when coincubated with anti-TF antibody. (3) Cancer metastasis was found in 22.2% of nude mice transplanted with A2780 cells, while in 88.9% of those transplanted with A2780/TF cells.

CONCLUSIONTF could promote the invasion and metastasis of human ovarian cancer cells through TF/FVIIa pathway.

Animals ; Cell Line, Tumor ; Cell Movement ; Cloning, Molecular ; Factor VIIa ; genetics ; physiology ; Female ; Genetic Vectors ; Humans ; Mice ; Mice, Inbred BALB C ; Mice, Nude ; Neoplasm Invasiveness ; Neoplasm Metastasis ; Neoplasm Transplantation ; Ovarian Neoplasms ; genetics ; pathology ; Thromboplastin ; genetics ; physiology ; Transfection ; Transplantation, Heterologous

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Inherited coagulation factor X deficiency caused by two novel mutations in factor X gene.

Wen-bin WANG ; Hong-li WANG ; Xue-feng WANG ; Qi-hua FU ; Rong-fu ZHOU ; Shuang XIE ; Yi-qun HU ; Zhen-yi WANG

Chinese Journal of Hematology.2004;25(9):519-522.

OBJECTIVETo explore the molecular mechanisms involved in a pedigree with inherited coagulation factor X (FX) deficiency.

METHODSThe activated partial thromboplastin time (APTT), prothrombin time (PT), FX activity (FX:C) and FX antigen (FX:Ag) test were adopted for phenotype diagnosis. All the 8 exons, intron/exon boundaries and the 5'untranslated regions (UTR) of the FX gene were amplified by polymerase chain reaction (PCR) from the genomic DNA extracted from the peripheral blood of the propositus. The PCR products were screened by direct sequencing. The mutation was confirmed by allele specific PCR (ASPCR).

RESULTSThe phenotype of the propositus was identified as FX deficiency (type II). Two novel FX gene mutations were detected in the propositus: one was a donor site splice mutation in intron 1 (IVS1 + 1G-->A), another was a missense mutation 1185G-->A in exon 8 (Arg347His).

CONCLUSIONThe FX deficiency of the propositus is caused by double heterozygous mutations IVS1 + 1G-->A and Arg347His.

Antigens ; genetics ; Base Sequence ; DNA Mutational Analysis ; Factor X ; genetics ; Factor X Deficiency ; genetics ; Female ; Heterozygote ; Humans ; Male ; Mutation ; Pedigree ; Young Adult

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