The clinical value of F-18 FDG PET/CT in patients with secondary hemophagocytic syndrome / 中国实验血液学杂志

The aim of this study was to investigate the role of F-18 fluoro-2-deoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) in diagnosis and prognostic evaluation of secondary hemophagocytic syndrome (HPS). A total of 11 secondary HPS patients examined with 18F-FDG-PET/CT were retrospectively analyzed. The diagnostic value of F-18 FDG PET/CT for malignancy detection was assessed. The values of maximum standardized uptake value (SUV(max)) in spleen (SUVS(p)) and in bone marrow (SUVBM) were measured to analyze their relationship with various laboratorial parameters and clinical outcome of secondary HPS patients. The results showed that 4 out of the 11 patients had malignancies, the sensitivity, specificity and diagnostic accuracy of F-18 FDG PET/CT for malignancy detection were 100%, 66.7% and 75% respectively, the SUV(max) of spleen and bone marrow showed no significant correlation with laboratorial parameters, a maximum SUVS(p) of 3.10 and a maximum SUVBM of 3.47 were the optimal cutoffs for predicting patients' outcome, the increased uptake of F-18 FDG in the BM and spleen were significantly associated with shorter survival time according to univariate analysis. It is concluded that 18F-FDG PET/CT may especially play an important role in diagnosis and predicting outcome of secondary HPS for the small sample size.

Immunophenotyping characteristics of AML and their correlation with the curative effects / 中国实验血液学杂志

This study was aimed to explore the immunophenotyping characteristics of acute myeloid leukemia (AML) and their correlation with the curative efficacy. The bone marrow or blood samples were collected from 516 patients with newly diagnosed AML, and their immunophenotypes were analyzed by flow cytometry. The results showed that (1) In 516 cases, the ratios of myeloid antigen expression were higher, as follows: MPO 95.0%, CD33 93.0%, CD13 88.8%, CD117 69.4%; and the expressions of CD14, CD15, CD64 and CD71 were lower, meanwhile 145 cases were accompanied with lymphocyte antigen expression, the ratios were as follows: CD7 21.5%, CD19 6.0%, CD2 0.78%, CD10 0.58% and CD20 0.58%; the positive expression rate of CD71 in M6 was 100%, and that of CD64 in M5 was the highest (30.2%); the overall positive rate of CD34 was 57.8%. (2) After first chemotherapy, the complete remission (CR) rate was 64.7%, CR rate of CD34(+) patients was lower than that of CD34(-) in M3 group (P = 0.019). The CR rate of CD34(+) patients was significantly lower than that of CD34(-) in non-M3 group (P = 0.002). The CR rate of CD19(+) patients was higher than CD19(-) (P = 0.028); the CR rate of CD7(+) patients was significantly lower than that of CD7(-) (P = 0.002); the CR rate of CD71(+) patients was lower than that of CD71(-) (P = 0.013); the CR rate of MPO(+) patients was higher than that of MPO(-) (P = 0.015). Between the CR rate of CD11b, CD13, CD33-positive and-negative group, the difference was not statistically significant (P > 0.05). It is concluded that the phenotype is a prerequisite for the diagnosis of AML, and can help to guide the clinical typing, selection of treatment protocols and evaluation of prognosis.

Expression of c/ebpα gene in MDS and its clinical significance / 中国实验血液学杂志

This study was aimed to investigate the expression of CCAAT/enhancer binding protein alpha gene (c/ebpα) in patients with myelodysplastic syndromes (MDS) and to explore the significance of c/ebpα in pathogenesis and progression of MDS. Real time quantitative reverse transcriptase polymerase chain reaction (RQ-PCR) method was used to detect the expression level of c/ebpα mRNA in bone marrow mononuclear cells (BMMNC) of 33 patients with MDS and 14 normal controls. The results showed that the expression level of c/ebpα mRNA in low-risk and high-risk MDS was significantly lower than that of normal controls (p < 0.01, p < 0.001, respectively), moreover, high-risk MDS showed lower c/ebpα mRNA expression compared with low-risk MDS (p < 0.05). c/ebpα mRNA expression level in MDS was not correlated with sex, age and peripheral blood cell amount, while the ratio of blast cells in bone marrow was in the c/ebpα mRNA low expression group significantly higher than that in the high expression group (p < 0.01). It is concluded that down-regulation of c/ebpα mRNA expression level has closely associated with the pathogenesis of MDS, the c/ebpα may be an important molecular biological marker of MDS; the degree of down-regulated c/ebpα has closely related to the progression of MDS.

Study on cytogenetic changes with relation to FAB classification in 397 patients with acute leukemias / 中国实验血液学杂志

The purpose of study was to investigate the cytogenetic abnormality of acute leukemias (AL), to analyze the relationship in the chromosomal abnormality and the AL FAB types, and to explore the impact of the chromosomal abnormalities on the prognostic factors of AL. The chromosome karyotypes of 397 patients with AL were analyzed by means of bone marrow short-term culture and G banding technique. The results showed that in 319 out of 397 patients, the chromosome karyotypes could be analyzed, and the chromosomal abnormality occurred in 175 patients (54.9%). In the patients with acute lymphocytic leukemia (ALL), acute myelogenous leukemia (AML) and acute mixed-lineage leukemia (AMLL), the chromosomal abnormality occurred respectively in 33 of 120 patients (27.5%), 129 of 252 patients (51.2%) and 13 of 25 patients (52.0%). Hyper-diploids, hypo-diploids and diploids occurred in 41 of 175 patients (23.4%), 22 of 175 patients (12.5%), and 112 of 175 patients (64.0%) respectively. In patients with AML the FAB type-associated chromosomal abnormality occurred in 69 of 129 patients (53.5%). It is concluded that chromosomal abnormalities exist in about 55% AL patients. Some special chromosomal abnormalities are cytogenetic characteristics of AL, and obviously correlated with AL FAB types, the combination of chromosomal detection with cytogenetics is useful for the diagnosis of AL, and the evaluation of therapeutic effects and prognosis.

Correlative study between JAK2 mutation and thrombosis in patients with myeloproliferative neoplasm / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the frequency and clinical implication of JAK2 mutation in patients with myeloproliferative neoplasm(MPN)and the correlation between the mutation and thrombosis.</p><p><b>METHODS</b>The clinical and laboratory data of 107 MPN patients was retrospectively analyzed. JAK2 mutation were detected with allele-specific polymerase chain reaction (AS-PCR) and sequencing. The significance of the mutation in disease diagnosis and molecular pathogenesis and the correlation between the mutation and thrombosis was analysed.</p><p><b>RESULTS</b>JAK2 mutation was detected in 71 (66.4%) and thrombosis in 34 (31.8%) of the 107 MPN patients. Thrombosis occurred in 34.8% (16/46) of polycythemia vera (PV), 32.6% (14/43) of essential thrombocythemia (ET), and 22.2% (4/18) of primany myelofibrosis (PMF) patients. The difference among the 3 groups was not significant (χ(2) = 0.96, P > 0.05). The frequency of thrombosis in JAK2(+) MPN patients (82.4%, 28/34) was higher than that in JAK2(-) MPN patients (17.6%, 6/34) (χ(2) = 5.71, P < 0.05). The frequency of thrombosis in MPN patients > 60 years was higher (41.5%, 27/65) than that in patients < 60 years (16.7%, 7/42) (χ(2) = 7.28, P < 0.01).</p><p><b>CONCLUSION</b>JAK2 V617F mutation occurs in significant percentage of Chinese patients with MPN. Patients with JAK2 mutation and older age are more succeptible to thrombosis. JAK2 mutation screening in patients with unknown thrombosis is helpful to reveal the underlying latent-MPN.</p>

Unrelated cord blood transplantation in adult patients with hematologic malignancies / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyse the engraftment, transplant-related complications and survival after unrelated cord blood transplantation (UCBT) in patients with hematologic malignancies.</p><p><b>METHODS</b>Twenty eight consecutive adult patients with hematological malignancies were treated with UCBT and 20 of them were advanced-stage diseases. Double or multiple UCB grafts were used for 18 patients, while single UCB graft for 10 patients. Myeloablative conditioning regimens were given to 26 cases and nonmyeloablative regimens to 2 cases. All patients were given a combination of cyclosporine (CsA) and mycophenolate mofetil (MMF) for graft-versus-host disease (GVHD) prophylaxis.</p><p><b>RESULTS</b>Median time to neutrophil engraftment (≥ 0.5 × 10(9)/L) in 26 patients was 18 (14 - 37) days and platelet engraftment (≥ 20 × 10(9)/L) in 22 patients was 30 (25 - 49) days. Chimerism was weekly assessed by PCR analysis of short tandem repeat (STR) sequences in whole blood or bone marrow and 22 cases were confirmed of fully donor chimeric from 7 to 21 days after transplantation. Eighteen cases developed acute GVHD, greater than grade II in 1, and 6 of 22 patients who survived more than 100 days developed limited chronic GVHD. Eighteen cases were alive in hematologic remission at a median follow-up of 9.5 (2.5 - 72.0) months. The probability of event-free survival at 3 years was 56.7%. Two cases relapsed and 8 of 10 cases died of transplant related complications.</p><p><b>CONCLUSIONS</b>UCBT could be safely and effectively used for adult patients with hematologic malignancies. Use of double UCB units is a strategy extending the feasibility of UCBT.</p>

Clinical significance of anticoagulant proteins detection in patients with thrombotic events / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the prevalence and the risk of natural anticoagulants such as plasma protein C (PC), protein S (PS) and antithrombin (AT) deficiency in thromboembolic patients with no evident acquired factors.</p><p><b>METHODS</b>Clotting assays on French STAGO autoanalyzer were used to detect the activity of plasma PC, PS and AT in 85 patients with thrombotic disease and 50 sex and age matched healthy controls.</p><p><b>RESULTS</b>Among the 85 enrolled patients (18 arterial and 67 venous thromboembolism), male to female ratio was 1.4 and the median age was 42 years (17-69). The activity of plasma PC, PS and AT in the pre-therapy thrombotic disease group, the thrombo-recurrence group, and the age < or = 45 years group were significantly lower than that is the healthy control group, the first thrombotic episodes group and the age > 45 years group respectively (P < 0.001, P < 0.01, P < 0.01). The overall deficiency rate of these three natural anticoagulants was 30.6%, PS deficiency was the commonest (10.6%), the second was PC deficiency (8.2%), AT deficiency and combined deficiency each accounted for 5.9%.</p><p><b>CONCLUSION</b>The PC, PS and AT protein deficiencies are frequent in Chinese thromboembolic patients, they are the independent risk factors for the thrombotic events and recurrence.</p>