Identification of a Novel CSNK2A1-PDGFRB Fusion Gene in a Patient with Myeloid Neoplasm with Eosinophilia / Journal of the Korean Cancer Association, 대한암학회지

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Identification of a Novel CSNK2A1-PDGFRB Fusion Gene in a Patient with Myeloid Neoplasm with Eosinophilia / Journal of the Korean Cancer Association, 대한암학회지

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Development and application of anti-human platelet and anti-thrombotic monoclonal antibodies / 白血病·淋巴瘤

Since its inception, anti-platelet antibodies have been an important tool for studying the interaction of platelets with blood components and blood vessels. At the same time, anti-platelet antibodies also play an important role in the detection and diagnosis of hemorrhagic and thrombotic diseases, and become a kind of powerful anti-thrombotic drugs. With the further understanding of the role of platelets in physiological hemostasis and pathological thrombosis, anti-platelet and anti-thrombotic antibodies also seek a balance between better anti-thrombotic effects and less bleeding side effects.

Clinical observation of desmopressin in the treatment of 15 patients with DDAVP / 中华血液学杂志

Objective@#To assess the significance of DDAVP use in the diagnosis and treatment of VWD.@*Methods@#An analysis of 15 VWD cases who referred to Hematology Division of First affiliated Hospital of Soochow University and treated with DDAVP from March 2016 to August 2018 was conducted. Efficacy and treatment response of DDAVP were monitored by observations of changes in factor Ⅷ procoagulant (FⅧ∶C) and von Willebrand Factor (VWF) related indicators before and 2 h after DDAVP injection.@*Results@#Of 15 cases with VWD, 7 males and 8 females with a median age of 23 (6-46) years, 7 of 9 type I VWD patients achieved complete response (CR) , 1 type 2A VWD case CR, 5 type 3 VWD ones no response (NR) . The VWF multimer analysis in 5 patients combined with other plasma VWF values were in accordance with the known diagnosis.@*Conclusions@#DDAVP was effective in most type 1 patients, and ineffective in some type 2 and almost all type 3 cases. It was helpful for diagnosis and subsequent treatment planning.

The comparative study between thromboelastography and conventional coagulation tests in ischemic cerebral vascular disease / 中华急诊医学杂志

Objective@#To explore the correlation and consistency between thromboelastography (TEG) and traditional coagulation tests (CCTs) in ischemic cerebral vascular disease (ICVD).@*Methods@#Totally 108 ICVD patients admitted to Nanyang Central Hospital from May 1 to October 31 2018 were enrolled. Patients’ TEG parameters (R value, K value, Angle value, MA value, CI value and G value) and CCTs parameters (PT, APTT, TT, and FIB) were collected and analyzed retrospectively. The Spearman correlation coefficient was used to explore the correlation between TEG and CCTs parameters, and Kappa (κ) to explore the consistency in determining the coagulation status of the patients. The ROC curve was used to analyze the predictive value of TEG parameters for abnormal results of CCTs, and the results of TEG and CCTs were comprehensively analyzed to evaluate the ability to predict the coagulation status of patients.@*Results@#(1) PLT was positively correlated with MA value and G value; PT and APTT were positively correlated with K value; TT was positively correlated with R value and K value; FIB was positively correlated with Angle value, MA value and G value. TT was negatively correlated with Angle value and CI value; FIB was negatively correlated with K value. (2) PT and MA values, PT and G values, FIB and MA values, FIB and G values were accordant in valuing the hypoxic state of ICVD patients. (3) PLT and Angle values, PLT and MA values, PLT and CI values, PLT and G values were accordant in assessing hypercoagulable status of ICVD patients; FIB and Angle values, FIB and MA values, FIB and CI value, and FIB and G value were consistent in evaluating the hypercoagulable state of ICVD patients. (4) For detecting TT>20 s, the AUC of K value and Angle value were 0.648, 0.651, respectively; For detecting FIB>4 g/L, the AUC of Angle value and MA value were 0.717 and 0.747, respectively; For detecting PLT>300×109/L, the AUC of MA value was 0.808 (all P<0.05).@*Conclusions@#There is weak correlation and consistency between TEG and CCTs parameters in ICVD patients. The TEG parameters have good predictive value in evaluating the abnormal results of CCTs, but cannot replace the CCTs. Combination of these two methods can better reflect the coagulation status of patients, so as to afford assistance.

The comparative study between thromboelastograpby and conventional coagulation tests in ischemic cerebral vascular disease / 中华急诊医学杂志

Objective To explore the correlation and consistency between thromboelastograpby(TEG)and traditional coagulation tests(CCTs)in ischemic cerebral vascular disease(ICVD).Methods Totally 108 ICVD patients admitted to Nanyang Central Hospital from May 1 to October 31 2018 were enrolled.Patients' TEG parameters(R value,K value,Angle value,MA value,CI value and G value)and CCTs parameters(PT,APTT,TT,and FIB)were collected and analyzed retrospectively.The Spearman correlation coefficient was used to explore the correlation between TEG and CCTs parameters,and Kappa(κ)to explore the consistency in determining the coagulation status of the patients.The ROC curve was used to analyze the predictive value of TEG parameters for abnormal results of CCTs,and the results of TEG and CCTs were comprehensively analyzed to evaluate the ability to predict the coagulation status of patients.Results(1)PLT was positively correlated with MA value and G value; PT and APTT were positively correlated with K value; TT was positively correlated with R value and K value; FIB was positively correlated with Angle value,MA value and G value.TT was negatively correlated with Angle value and CI value; FIB was negatively correlated with K value.(2)PT and MA values,PT and G values,FIB and MA values,FIB and G values were accordant in valuing the hypoxic state of ICVD patients.(3)PLT and Angle values,PLT and MA values,PLT and CI values,PLT and G values were accordant in assessing hypercoagulable status of ICVD patients; FIB and Angle values,FIB and MA values,FIB and CI value,and FIB and G value were consistent in evaluating the hypercoagulable state of ICVD patients.(4)For detecting TT＞20 s,the AUC of K value and Angle value were 0.648,0.651,respectively;For detecting FIB＞4 g/L,the AUC of Angle value and MA value were 0.717 and 0.747,respectively; For detecting PLT＞ 300× 109/L,the AUC of MA value was 0.808(all P＜0.05).Conclusions There is weak correlation and consistency between TEG and CCTs parameters in ICVD Patients.The TEG parameters have good predictive value in evaluating the abnormal results of CCTs,but cannot replace the CCTs.Combination of these two methods can better reflect the coagulation status of patients,so as to afford assistance.

The diagnostic value of PLASMIC score in the discrimination between thrombotic thrombocytopenic purpura and disseminated intravascular coagulation / 中华血液学杂志

Objective@#PLASMIC score was evaluated its value in differential diagnosis between the patients with thrombotic thrombocytopenic purpura (TTP) and those with disseminated intravascular coagulation (DIC) .@*Method@#Twenty-four patients with TTP and 41 cases with DIC were retrospectively analyzed in this study. The platelet count, average red blood cell volume, indirect bilirubin, creatinine and prothrombin time international normalised ratio were collected, and then PLASMIC scores were calculated.@*Results@#According to the risk classification of PLASMIC score, three (12.5%) TTP patients had moderate risk, and the rest 21 (87.5%) cases had high risk. In DIC patients, 92.7% cases were in low risk group, 4.9% at moderate risk, and only one case had high risk. Of these 65 patients, the sensitivity and the specificity to TTP of the high risk of the scoring system were 87.5% and 97.6%, respectively.@*Conclusion@#The patients with high risk of PLASMIC score correlated well with clinical TTP diagnosis. The scoring system showed to be an excellent diagnostic model to distinguish TTP patients from those with DIC.

α1-antitrypsin Pittsburg mutations: report of two cases in the same family / 中华血液学杂志

Objective@#To analyze the clinical and laboratory abnormalities of two patients with α1-antitrypsin (α1-AT) Pittsburgh in a family and review the literatures.@*Methods@#Both plasma clotting time and factor activities were performed using clotting or substrate methods. Platelet aggregation was evaluated using an optical aggregometer. The serum protein electrophoresis was performed on Sebia HYDRASYS by using Agarose gel. The exons of α1-AT were amplified by using polymerase chain reaction (PCR) and then sequenced and compared with NCBI GenBank records.@*Results@#The proband had several ruptures of corpus luteum and bleeding after operation, while her daughter had no bleeding history. Both of them showed prolonged coagulation tests which could not be corrected by mixing with the normal plasma. They also showed low levels of plasma coagulation factors, undetected protein C and S activity and abnormal bands of α1-globulin. The results of gene sequencing demonstrated that they were heterozygous for g.T17132G (p.Met358Arg) mutation of α1-antitrypsin gene (NG_008290.1) .@*Conclusions@#Comparing with the data of previously reported cases, our results confirmed the obvious abnormality of coagulation test and the discrepancy of bleeding tendency of α1-antitrypsin Pittsburgh patients, and suggested that the rupture of corpus luteum would be a specific characteristic in women of child-bearing age.

A Study on the establishment of immune thrombocytopenia model induced by anti-platelet GPⅠbα antibodies / 中华血液学杂志

Objective@#To establish primary immune thrombocytopenia (ITP) animal model induced by anti-platelet membrane glycoprotein GPⅠbα antibodies AN51 and R300.@*Methods@#Twenty guinea pigs (6-8 week) were divided into 4 groups. Five guinea pigs in each group were intravenously injected with different doses of AN51 (0.05, 0.1, 0.2 μg/g) and 0.2 μg/g IgG as control. The whole blood was collected from inner angular venous plexus. Platelets number was determined by an automated cell counter and Swiss-Jim method. Then, the similar protocol was used to establish ITP nude mice model by intraperitoneal injection of different concentrations of anti-platelet GPⅠbα antibody R300, respectively.@*Results@#①Five minutes after intravenous injection of AN51 at 0.05, 0.1 and 0.2 μg/g, the platelet counts of guinea pigs reduced about 0-5%, 50%-60% and 70%-80% compared to the control group, respectively. The difference was statistically significant (P<0.01) . ②Six hours after intraperitoneal injection of R300 at 0.05, 0.1, 0.2 μg/g, the platelet counts of nude mice decreased about 20%-30%, 60%-70% and 80%-90% compared to the control group, respectively. The difference was statistically significant (P<0.01) . The nude mice, injected 0.2 μg/g R300 once a day for 2 weeks, showed typical ITP clinical manifestations including large number of petechiaes or ecchymoses on limbs, head and abdomen.@*Conclusion@#AN51 at 0.2 μg/g and R300 at 0.2 μg/g could establish stable ITP model in guinea pigs and nude mice respectively.

Detection and analysis of plasma VWF levels in healthy Chinese by automatic standardized assay / 中华血液学杂志

Objective@#To explore the normal range of plasma VWF levels of healthy Chinese and to analyze the influencing factors to VWF level.@*Methods@#To detect the levels of von Willebrand factor antigen (VWF∶Ag) , von Willebrand factor ristocetin cofactor activity (VWF∶Rco) , von Willebrand factor collagen binding activity (VWF∶CB) , and the factor Ⅷ coagulation activity (FⅧ∶C) by using fully automatic and standardized testing instruments and matching reagent in 70 healthy Chinese. The effects of age, ABO blood type, gender and region were also analyzed. Meanwhile, 8 standard plasma samples (2 normal subjects, 6 cases of type 2 VWD) confirmed by NIBSC were tested for VWF values.@*Results@#① In 70 cases of healthy Chinese, the mean value of plasma VWF∶Ag, VWF∶Rco and VWF∶CB were (95.4±44.9) %, (105.9±35.4) % and (89.8±28.4) %, respectively; the ratio of VWF∶Rco/VWF∶Ag and VWF∶CB/VWF∶Ag was 1.18±0.25 and 1.03±0.29, respectively. ②There was no statistical significance in plasma VWF values between the age ≥30 years and <30 years group (P>0.05) . ③The VWF∶Rco, VWF∶CB of type O blood group were lower than that of non-O group (t=2.074, P=0.042; t=3.949, P=0.001) , but there was no statistical significance in VWF∶Ag, VWF∶Rco/VWF∶Ag, VWF∶CB/VWF∶Ag between the two groups (P>0.05) . ④There was no significant difference in VWF values between male and female groups (P>0.05) . ⑤The VWF∶Ag, VWF∶CB of the northern population (North area of Huaihe River) group were higher than that of southern population (Suzhou area) group (t=4.525, P=0.001; t=3.214, P=0.002) , but VWF∶Rco/VWF∶Ag, VWF∶CB/VWF∶Ag were lower than that of southern population group (t=6.373, P=0.001; t=2.902, P=0.005) , and there was no significant difference in VWF∶Rco between the two groups (t=1.598, P=0.115) . ⑥The VWF values of 8 standard plasma samples were in accordance with the known diagnosis.@*Conclusions@#A more integrate plasma VWF levels of healthy Chinese people were obtained for the first time by using fully automatic and standardized testing instruments. It was also found that ABO blood group and region had a significant impact on the level of VWF, while the age and gender had no significant effect.

Circulating microRNAs as biomarker for venous thromboembolism diagnosis / 中华检验医学杂志

Venous thromboembolism ( VTE) remains the third most common cardiovascular disease with a vague pathogenesis.Conventional biomarkers exhibit poor performance in the diagnosis, surveillance and prognosis of VTE.MicroRNAs ( miRNAs ) are a class of evolutionarily conserved small non-coding RNAs that are involved in the regulation of gene expression and protein translation An array of experimental studies has shown the importance of miRNAs for disease initiation/progression.Circulating miRNAs are found in plasma, serum and other body fluids in an apparently stable form.Recent evidence revealed that circulating miRNAs, a novel family of regulatory molecules, emerge as a promising class of biomarkers in many cardiovascular diseases, malignancies as well as VTE.This review describes current understanding of miRNA biogenesis and the origins and types of circulating miRNAs and gives an outline of recent work on circulating miRNAs as well as its challenges and perspectives of the clinical utility of circulating miRNA in VTE.

A single-center clinical study of 22 patients with acquired hemophilia / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the feature of underlying disorders, clinical symptoms, diagnosis and treatment strategies of patients with acquired hemophilia(AH).</p><p><b>METHODS</b>The clinical data and laboratory tests results of 22 patients with AH from March 2010 to June 2014 were retrospectively analyzed.</p><p><b>RESULTS</b>A total of 22 patients with AH were enrolled in our study, including 20 patients diagnosed as acquired hemophilia A (AHA) and 2 as acquired hemophilia B (AHB). Among the AHA patients, there were 10 males and 10 females with the median age of 37.5 (range, 2-95) years old. The median activity of FVIII (FVIII:C) of the 20 AHA patients was 1.9% (0.5%-39.0%). Soft tissue hematoma (80.0%) and subcutaneous bleeding (75.0%) were the most common clinical symptoms. Two male children were diagnosed as AHB (age 1 and 3 years old, respectively) with mild bleeding symptoms, and the activities of FIX (FIX:C) were 5.0% and 16.0%, respectively. In addition, an underlying disorder was found in 7 patients (31.8%). In laboratory testing, all patients had prolonged APTT, normal PT, decreased FVIII:C or FIX:C, positive antibody screening test or antibody titer (2-32 BU), and negative for lupus anticoagulant and anticardiolipin antibody. Nineteen out of 20 patients were treated with blood products to stop acute bleeding episodes. Corticosteroid alone was applied to 7 patients, corticosteroid combined with other immunosuppressive agents to 11 patients, rituximab to 3 patients. Nineteen patients responded well to hemostatic treatment, except 1 patient who died of fatal bleeding. The FVIII:C of 8 patients increased to a normal level with the median time of 42.5(21-145) days. After treatment, the activity of FIX:C of the 2 AHB patients achieved 35% and 24% in 48 and 60 days, respectively.</p><p><b>CONCLUSION</b>Acquired hemophilia is not an uncommon disease in clinical practices, which can occur in people of all ages. AH is a bleeding disorder with heterogeneous characteristics. Compared with adult, the clinical symptoms of children patients were mild, which lead to underdiagnosis.</p>

Use of recombinant factor VIIa in uncontrolled gastrointestinal bleeding after hematopoietic stem cell transplantation among patients with thrombocytopenia

Background and Objective: Recombinant-activated factor VII [rVIIa] is a vitamin K-dependent glycoprotein that is an analog of the naturally occurring protease. It has an off-label use to control life-threatening bleeding that is refractory to other measures and was shown to decrease transfusion requirements. Gastrointestinal [GI] bleeding is a severe complication following hematopoietic stem cell transplantation [HSCT] in patients with thrombocytopenia, while hemostatic measures based on antifibrinolytic or transfusion therapy may not always be successful. The present study investigated the treatment with rFVIIa in severe GI bleeding among thrombocytopenia patients undergoing HSCT

Methods: rFVIIa was given as a single dose of 60 microg/kg in patients with GI bleeding following hematopoietic stem cell transplantation [HSCT]

Results: Among all patients enrolled, 12 [75%] of 16 patients obtained a response, of which 5 achieved a complete response and 7 achieved a partial response. The 4 remiaing patients [25%] had no response. Nine patients [56.3%] died in a follow-up of 90 days. No thromboembolic events wereassociated with the drug administration occurred

Conclusions: Our study showed that rFVIIa may represent an additional therapeutic option in such cases

Outcomes of allogeneic hematopoietic stem cell transplantation for 18 patients with paroxysmal nocturnal haemoglobinuria / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the outcomes of allogeneic hematopoietic stem cell transplantation（allo-HSCT）for paroxysmal nocturnal haemoglobinuria（PNH）and aplastic anemia（AA）- PNH syndrome.</p><p><b>METHODS</b>The clinical data of 18 PNH or AA-PNH patients, including 4 classic PNH and 14 AA-PNH, received allo-HSCT from Dec 2007 to Feb 2015 were analyzed retrospectively. Nine patients received HLA-haploidentical donor HSCT（1 patient received salvage HLA-haploidentical donor HSCT after the graft failure of double cord blood transplantation）, 7 patients received HLA-identical sibling donor HSCT, and 2 HLA-identical unrelated donor HSCT. The conditioning regimens were as follow: 13 patients received modified BU/CY- based regimens, 5 non- myeloablative regimens ［fludarabine （Flu） + antithymocyte globulin（ATG）+ cyclophosphamide（CY）or busulfan（BU）］. Prophylaxis for graft- versushost disease（GVHD）: the patients with HLA-identical sibling donor received cyclosporine（CsA）plus short-term methotrexate（MTX）, the patients with HLA -haploidentical donor or HLA-identical unrelated donor received CsA or tacrolimus（FK506）+ mycophenolate mofetil（MMF）+ short- term methotrexate （MTX）.</p><p><b>RESULTS</b>All patients were engrafted successfully（1 patient engrafted by haploidentical donor after the graft failure of double cord blood transplantation）. The median days of neutrophils（ANC）above 0.5 × 109/L and platelets （PLT） more than 20 × 10⁹/L were 11（10- 26）days and 15（11- 120）days, respectively. Three patients（17.6%）developed acute GVHD（aGVHD）, 2 for grade Ⅱ aGVHD, 1 for grade Ⅳ aGVHD. Of 16 patients, 2 occurred limited chronic GVHD（cGVHD）. After a median follow-up of 14.6（2.0-86.7）months, 3 patients（17.6%）died, out of which one died of severe aGVHD, one died of severe pulmonary infection, one pulmonary infection with transplant- associated thrombotic microangiopathy. The 5- year estimated disease free survival was（80.5 ± 10.2）%. No patient relapsed.</p><p><b>CONCLUSION</b>Allo-HSCT is an effective and curable therapy for PNH or AA-PNH with improved prognosis, and offers a valid therapeutic option for these patients before humanized monoclonal antibody against C5 are widely used clinically.</p>

Hypofibrinogenemia caused by long-term administration of hemocoagulase: three cases report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>A first report of 3 patients who developed hypofibrinogenemia due to long-term administration of hemocoagulase.</p><p><b>METHODS</b>The clinical data of three patients with hypofibrinogenemia due to long-term administration of hemocoagulase were analyzed, and the related literature was reviewed.</p><p><b>RESULTS</b>Case 1, a two-year old girl, had liver traumatic rupture and then treated with massive transfusion and fibrinogen infusion in addition to intravenous recombinant factor VIIa (two times) and hemocoagulase (2 U/d). The liver wound bleeding was soon stopped. However, her plasma fibrinogen level decreased to 0.12 g/L after continuous administration of hemocoagulase for 18 days. Case 2, a three-year old boy, had liver traumatic rupture and was treated with surgical repair, and then received hemocoagulase (2 U/d). On the 8th day, a large amount of blood was found to exude from abdominal cavity drainage tube and indwelling venous catheter, and his fibrinogen dropped to 0.24 g/L. Case 3 was a 45 year-old man who underwent a total mandibular resection because of malignant tumor, and he was given hemocoagulase (4 U/d). A continuous blood oozing was noted from his operation incision, and his fibrinogen level decreased to 0.25 g/L. All the three patients'plasma fibrinogen levels and coagulation tests returned to normal ranges after discontinuation of hemocoagulase administration and supplement of fibrinogen, and the bleeding stopped in cases 2 and 3.</p><p><b>CONCLUSION</b>Long-term use of hemocoagulase could induce hypofibrinogenemia and severe bleeding.</p>

The clinical studies of 51 patients with thrombotic thrombocytopenic purpura / 中华血液学杂志

<p><b>OBJECTIVE</b>To comprehensively analyze the clinical characteristics, treatment strategies and outcome of patients with thrombotic thrombocytopenic purpura (TTP).</p><p><b>METHODS</b>A retrospective survey of 51 TTP patients confirmed in our database. Relevant statistical analyzes were performed by GraphPad Prism 5 software.</p><p><b>RESULTS</b>51 cases of patients with acquired TTP were identified as idiopathic TTP. In our study, only 18 cases (35.29%) had typical pentalogy of TTP, where thrombocytopenia (100.00%), microangiopathic hemolytic anemia (92.16%) and neurologic abnormalities (88.24%) were more common than fever (72.55%) and renal abnormalities (70.59%). Plasma ADAMTS13 activity was detected in 37 patients with TTP with ADAMTS13 deficiency confirmed in 31 patients (83.78%). Plasma exchange with response of 72.3% was still the preferred strategy in TTP with individuation. Among 36 survival TTP patients, 8 patients (22.22%) relapsed. 15 patients (29.41%) died in our study. The mean ages of responders and deaths were of (37.5±14.5) and (50.1±18.9) respectively; whereas total bilirubin level of responders and deaths were of (43.3±23.5)μmol/L and (63.7±39.7) μmol/L respectively, the differences were statistically significant. Conversely, body temperature, WBC, HGB, PLT, serum creatinine and LDH showed no significant differences (P>0.05).</p><p><b>CONCLUSION</b>The diagnosis of TTP was based on comprehensive analysis of clinical manifestations. Plasma ADAMTS13 activity test had a higher clinical practical value. The therapeutic alliance with corticosteroids, immunosuppressive agents and Rituximab significantly improved its outcome. The age and high total bilirubin level at onset were associated with less sensitive to plasmapheresis and poor prognosis.</p>

Clinical studies of 162 patients with von Willebrand disease / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyse the epidemiological feature, clinical characters and therapeutic regimens for von Willebrand disease(VWD).</p><p><b>METHODS</b>The clinical data and laboratory tests results of 162 VWD patients in our center were analyzed.</p><p><b>RESULTS</b>There were 76 males and 86 female among these patients with the mean age of 7.2(2.0-41.0) and 20.7(5.0-48.0) years, respectively. 86 patients (53.1%) were identified to be type 1 VWD, 34 patients (21.0%) type 3 VWD and 42 patients (25.9%) type 2 VWD. Among type 2 VWD patients, 33 patients were type 2A, 4 patients type 2M, 5 patients type 2B. Eighty-seven patients (53.7%) had a definite family history of bleeding tendency. The most common and specific bleeding symptoms were easy bruising (61.7%), epistaxis (53.7%), prolonged bleeding after surgery or minor injury (53.1%). Menorrhagia (66.3%) was common in female patients. The analysis of Vicenza bleeding scores in all patients showed that only 56(34.6%) patients had abnormal bleeding scores. FVIII/VWF concentrates and cryoprecipitate were applied to 45 patients (27.8% ), Desmopressin (DDAVP) to 8 patients. Eight female patients need oral contraceptives jointly to control menorrhagia. Hysterectomy had to be performed in 2 female patients with VWD.</p><p><b>CONCLUSION</b>VWD was a common congenital bleeding disorder with heterogeneous characters, it was necessary to screen, identify, classify accurately this disease in order to supply to effectively individualized treatment.</p>

Performance evaluation of PL-11 platelet analyzer / 中华检验医学杂志

Objective To evaluate and report the performance of PL-11 platelet analyzer.Methods Intravenous blood samples anticoagulated with EDTA-K2 and sodium citrate were tested by the PL-11 platelet analyzer to evaluate the intra-assay and inter-assay coefficient of variation (CV),carry-over rate,accuracy,linearity of the PL-11 platelet analyzer.Platelet aggregation rate of sodium citrate-anticoagulated fasting venous blood collected from 30 physical examinees in outpatient department of The First Affiliated Hospital of Soochow University between February and July,2012 was detected by the PL-11 platelet analyzer and MPG-3E multifunctional double channel blood coagulation analyzer,respectively.The correlation was detected between the PL-11 platelet analyzer and MPG-3E multifunctional double channel blood coagulation analyzer.Results All the parameters were conformed to the standard of Clinical Laboratory Improvement Amendment 88.Both of the intra-assay and inter-assay CV values were less than 5％ ; carry-over rate was less than 1％ ; the accuracy and the linearity was excellent correlated to the result of photoelectric turbidimetry (R2 =0.9439).Conclusions PL-11 platelet analyzer can directly use whole blood dynamics to analyze platelet aggregation process and quantitatively analyze the various components of blood cells (including platelets and red blood cells),the result reports are accurate and reliable.

The value of anti-von Willebrand factor antibody in the mechanism of idiopathic thrombotic thrombocytopenic purpura / 中华检验医学杂志

Objective This study is aimed at determining whether anti-von Willebrand factor (VWF) autoantibodies are present in the plasma of idiopathic thrombotic thrombocytopenic purpura (TTP) patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies,and at examining whether murine monoclonal antibodies (mAbs) against human VWF decrease the susceptibility of VWF to ADAMTS13 in vitro.Methods Anti-VWF autoantibodies and ultralarge VWF (UL-VWF) multimers were measured in plasma samples of 53 adult patients with idiopathic TTP by enzyme-linked immunosorbent assay and sodium dodecylsulphate-agarose gel electrophoresis,respectively.Moreover,the effects of eight murine mAbs to different human VWF domains on VWF cleavage by ADAMTS13 were evaluated under fluid shear stress and static/denaturing conditions,respectively.Results Anti-VWF antibodies and UL-VWF multimers were detected in two TTP patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13antibodies.The SZ34,an anti-VWF mAb,inhibited VWF proteolysis mediated by ADAMTS13 under flow,but not static conditions.Conclusion Anti-VWF antibody may be one of the causes of idiopathic TTP with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies.