Effect of Circulating Plasma Cells on the Prognosis of Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#to analyze the effect of circulating plasma cells(CPC) on the prognosis of patients with multiple myeloma(MM) in the era of new drugs, and to explore the new definition standard of primary plasma cell leukemia(pPCL).@*METHODS@#The clinical data of 321 patients with newly diagnosed MM and 21 patients with pPCL admitted to our hospital from January 2014 to May 2022 were retrospectively analyzed. According to the proportion of CPC in peripheral blood smears, all patients were divided into 4 groups: CPC 0% group(211 cases), CPC 1%-4% group(69 cases), CPC 5%-19% group(41 cases) and CPC≥20% group(21 cases). The clinical features of patients in each group were compared and the prognosis fators was analyzed.@*RESULTS@#The median OS of the four groups were 44.5,21.3,24.6 and 12.8 months, respectively. Among them, 295 patients(86.3%) were treated with new drugs, and the median OS of the four groups were not reached, 26.7, 24.6 and 14.9 months, respectively. As the survival curves of CPC 5%-19% group and CPC≥20% group were similar, the patients were divided into CPC<5% group and CPC≥5% group, the median OS of CPC<5% group was better than that in CPC≥5% (43.5 vs 22.3 months, P<0.001). In addition, the median OS of patients in the CPC 1%-4% group was also significantly lower than that in the CPC 0% group and similar to that in the CPC≥5% group. Multivariate analysis showed that 1%-4% CPC was an independent risk factor for the OS of patients with CPC<5%. The patients with CPC<5% were stratified by R-ISS staging, and the OS of R-ISS stage Ⅰ or stage Ⅱ with 1%-4% CPC was similar to that of R-ISS stage Ⅲ. The newly defined pPCL patients showed increased tumor load and obvious invasive characteristics. Multivariate analysis showed no independent prognostic factors for pPCL, and high-risk cytogenetic abnormalities(HRCA) had no significant effect on the prognosis.@*CONCLUSION@#The validity of IMWG's new pPCL definition standard was verified, and it was found that the survival of MM with 1%-4% CPC also is poor and the prognosis is very close to pPCL. In addition, the newly defined pPCL has unique clinical and biological characteristics.

Correlation between D-Dimer/Fibrinogen Ratio and Bleeding in Patients with Newly Diagnosed Acute Promyelocytic Leukemia / 中国实验血液学杂志

OBJECTIVE@#To further explore the better indicators for predicting the degree of bleeding associated with newly diagnosed acute promyelocytic leukemia (APL).@*METHODS@#A total of 131 patients with newly diagnosed APL were classified according to WHO bleeding scales before treatment and divided into two groups: scales 0, 1 and 2 were included in no severe bleeding group, scales 3 and 4 were included in severe bleeding group. The information of the patients were collected, including sex, age, hemoglobin (Hb), white blood cell (WBC) count and platelet (PLT) count, peripheral blood lymphocyte percentage (LYMPH%), peripheral blood monocyte percentage (MONO%), percentage of leukemic cells in pripheral blood and bone marrow, prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB) levels, D-dimer (D-D), D-dimer/fibrinogen ratio (DFR).@*RESULTS@#Among 131 patients, 110 were classified as no severe bleeding, and 21 were severe bleeding. The results of univariate analysis showed that patients with severe bleeding had significantly higher percentage of leukemic cells in pripheral blood, WBC, D-D, and DFR, as well as longer PT and lower LYMPH%, compared to those with no severe bleeding. Multivariate analysis revealed that DFR (OR =1.054, 95%CI : 1.024-1.084, P < 0.001) and percentage of peripheral blood leukemic cells (OR=1.026, 95%CI: 1.002-1.051, P =0.033) were independent risk factors for severe bleeding. The area under ROC curve (AUC) of peripheral blood leukemic cells, D-D and DFR were 0.748, 0.736 and 0.809, respectively. There was no statistical difference between the peripheral blood leukemic cells and D-D in diagnostic efficacy (P =0.8708). Compared with D-D, DFR had a higher predictive value (P =0.0302). The optimal cut-off value of DFR was 16.50, with a sensitivity of 90.5% and a specificity of 70.0%.@*CONCLUSION@#DFR has a significant advantage in predicting the degree of bleeding associated with newly diagnosed APL. The greater the DFR value, the heavier the degree of bleeding. The risk of severe or fatal bleeding increases when DFR is greater than 16.50.

Relationship between Iron Metabolism and Platelet Parameters in Patients with Iron Deficiency Anemia / 中国实验血液学杂志

OBJECTIVE@#To investigate the relationship between iron metabolism and thrombocytosis.@*METHODS@#iron metabolism indexes , erythrocyte and platelet parameters of iron deficiency anemia patients with thrombocytosis were collected, the correlation of platelet parameters with iron metabolism indexes and erythrocyte parameters was analysed; the difference in erythrocyte，platelet and iron parameters between severe anemia group (hemoglobin<60 g / L) and mild and moderate anemia group (hemoglobin≥60 g / L) were compared; the changes of platelet count before and after treatment were observed.@*RESULTS@#There was a significant negative correlation between serum iron and platelet count (r=-0.404,P<0.01).Serum iron negatively correlated with platelet crit(r=-0.288,P<0.05). Transferrin saturation negatively correlated with platelet count and platelet crit(r=-0.353,P<0.01;r=-0.271, P<0.05).Serum ferritin and total iron binding capacity revealed no significant relation with any platelet parameters.Hemoglobin level , hematocrit and mean corpuscular hemoglobin concentration negatively correlated with platelet count(r=-0.239,P<0.05;r=-0.250,P<0.05;r=-0.339,P<0.01).There were differences in iron metabolism indexes and platelet parameters between mild to moderate anemia group and severe anemia group. After treatment, the platelet count decreased or was normal.@*CONCLUSION@#The important iron metabolism indexes affecting platelet count are serum iron and transferrin saturation,that is,the severer iron deficiency, the higher platelet count.Patients with more severe and hypochronic anemia has higher platelet count.

Expressions of SLC22A14 and SPAG6 proteins in the ejaculated sperm of idiopathic asthenozoospermia patients / 中华男科学杂志

Objective@#To investigate the expressions of solute carrier family 22 member 14 (SLC22A14) and sperm-associated antigen 6 (SPAG6) in the sperm of idiopathic asthenospermia men.@*METHODS@#We collected semen samples from 50 idiopathic asthenozoospermia patients and another 50 normal sperm donors, purified the sperm by discontinuous density centrifugation on Percoll gradients, and then determined the mRNA and protein expressions of SLC22A14 and SPAG6 by RT-PCR and Western blot, respectively.@*RESULTS@#Compared with the normal controls, the idiopathic asthenozoospermia patients showed significantly decreased mRNA expressions of SLC22A14 (0.77 ± 0.08 vs 0.53 ± 0.10, P<0.01) and SPAG6 (0.78 ± 0.09 vs0.52 ± 0.10 , P<0.01) and protein expressions of SLC22A14 (0.80 ± 0.09 vs 0.55 ± 0.10 , P<0.01) and SPAG6 (0.78 ± 0.09 vs 0.56 ± 0.09, P<0.01).@*CONCLUSIONS@#T The expressions of SLC22A14 and SPAG6 are reduced in the sperm of the patients with idiopathic asthenospermia, which may be one of the important causes of asthenospermia.

Expression and procoagulant activity of phosphatidylserine on the normal blood cells / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the expression and procoagulant activity of phosphatidylserine (PS) on the normal peripheral blood cells of adults.</p><p><b>METHODS</b>Normal peripheral blood samples were collected from 10 healthy volunteers (5 ml from each volunteer), platelets, neutrophils, lymphocytes and erythrocytes were isolated. The expression and procoagulant activity of PS on normal blood cells were identified by flow cytometry, inhibition test with lactadherin as PS probe and coagulation anticoagulant, respectively.</p><p><b>RESULTS</b>There was PS expression on a few normal blood cells (9.1%, 5.4%, 3.9% and 3.2% in platelets, neutrophils, lymphocytes and erythrocytes, respectively). The PS on these normal blood cells in vitro showed significant procoagulant activity. The plasma recalcification time was shortened by 47%, 36.5%, 25% and 12.5% by platelets, neutrophils, lymphocytes and erythrocytes, respectively; the formation of factor Xa (through both intrinsic and extrinsic pathways) and thrombin was also increased by 13% - 26% by platelets, neutrophils, lymphocytes and erythrocytes, respectively.</p><p><b>CONCLUSION</b>The PS on normal blood cells in vivo may play a crucial role in the coagulation cascade.</p>

Synergistic effects of different types of smoking and other risk factors on risk of hepatocellular carcinoma in Xiamen, China / 中华流行病学杂志

Objective To evaluated the independent effects of different types of smoking exposure along with multiple risk factors for hepatocellular carcinoma (HCC) and determined whether the magnitude of smoking was modified by other risk factors, both in men and women.Methods We conducted a case-control study in Xiamen China. 345 HCC patients and 961 healthy control subjects were personally interviewed for several HCC risk factors. Multivariate logistic regression analysis was performed to estimate the adjusted odds ratio (AOR) and 95% confidence interval (CI) for each potential risk factor. Results Cigars and pipes were not related to HCC among non-cigarette smokers. However, passive smoking exposure was associated with HCC in women:AOR, 2.35 (95%CI: 1.19-4.07). Regular cigarette smoking was associated with HCC in men: AOR,2.27 (95% CI: 1.14-3.31). Cigarette smoking and chronic infection of hepatitis B virus showed positive additive model interactions in men: RERI(relative excess risk due to interaction) was 98.70and AP (attributable proportion due to interactions) was 81.0%. Data on cigarette smoking with high AFB1-albumin adducts in women showed that the RERI was 2.69 and AP was 50.0%. Conclusion We concluded that sex differences were seen in HCC relationship with cigarette smoking. Controlling of exposure to smoking might be a prudent approach to the prevention of HCC, especially in patients with chronic viral hepatitis infections.

Gene analysis of five inherited factor V deficiency cases / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify gene mutations involved in five cases of inherited factor V (FV) deficiency.</p><p><b>METHODS</b>Activity of FV was determined by one-stage clotting assay using FV-deficiency plasma, and FV antigen by an ELISA assay. All the exons and exon-intron boundaries of FV gene were amplified by PCR and then DNA sequencing. Restriction enzyme analysis was used to analyze the probands, their family members and healthy volunteers.</p><p><b>RESULTS</b>Both activity and antigen of FV in the 5 patients were extremely lower compared with that of normal mixed plasma. Six mutations were identified in these 5 patients, G69969T (G2079V), C45533T (R712Ter), C46796T (R1133Ter), G45366A (C656Y), C46253T (R952C) and G16088C (D68H), the latter three were novel mutations reported for the first time and the C46253T (R952C) was the first missense mutation reported in B domain. The result of sequencing or restriction enzyme analysis showed that the three novel missense mutations were not caused by single nucleotide polymorphisms.</p><p><b>CONCLUSION</b>Gene mutations in 5 type I inherited FV deficiency of patients including 2 nonsense mutations and 4 missense mutations identified which led to the instability of FV protein and the reducing of FV: Ag in the plasma.</p>

Proteus syndrome with a giant hemangiomas in the spleen associated with chronic DIC--two case report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestations, pathologic features and laboratory findings in two Proteus syndrome patients with giant hemangiomas in the spleen and chronic DIC.</p><p><b>METHODS</b>Ultrasound imaging and magnetic resonance imaging (MRI) were used for analysing the characteristics of the giant hemangiomas in the spleen. The spleen specimen was examined pathologically for the feature of the hemangioma. Homostatic tests were performed by routine laboratory methods.</p><p><b>RESULTS</b>Two Proteus syndrome patients with giant hemangiomas in the spleen causing chronic DIC (Kasabach-Merritt syndrome) were first reported. They were recovered after splenectomy.</p><p><b>CONCLUSION</b>Proteus syndrome when accompanied giant hemangioma could cause chronic DIC. Significantly decreased plasma fibrinogen level in this case might be helpful for the differential diagnosis from DIC caused by other diseases.</p>

Clinical and molecular study on Fechtner syndrome--case report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify clinical and laboratory abnormalities and genetic defect of Fechtner syndrome in a Chinese family.</p><p><b>METHODS</b>The characteristic morphological features of platelets and leukocytes were examined on blood smears with Wright's-Giemsa staining and ultrastructure of platelet and leukocyte were investigated under electron microscope. Genomic DNA was isolated from peripheral blood of the proband and 9 members of his family. All the exons and exon-intron boundaries of the MYH9 gene were amplified by PCR followed by direct sequencing.</p><p><b>RESULTS</b>Patients presented the characteristic clinical features including macrothrombocytopenia, leukocyte inclusions and/or hereditary nephritis. A heterozygous C to T mutation was found in the proband and three members of his family at nucleotide 5981 in exon 40 of MYH9 gene, resulting in a nonsense mutation which encoded truncated protein due to premature termination at the Arg 1933 codon.</p><p><b>CONCLUSION</b>It is the first report of a Chinese family with Fechtner syndrome. The Arg (CGA) 1933--> stop (TGA) nonsense mutation in MYH9 gene is a causative genetic defect.</p>