Venetoclax plus cyclophosphamide and cytarabine as induction regimen for adult acute myeloid leukemia.

Background: The efficacy of induction chemotherapy (IC) for acute myeloid leukemia (AML) has improved significantly with the application of targeting drugs. Our previous study showed that a 4-day IC regimen of cyclophosphamide (CTX) and Ara-C [CA (4 + 3)] achieved similar complete remission (CR) rate (80%) compared with the traditional 7-day regimen, and the survival rate appeared to be better. Methods: In this pilot study, we further shortened the CA regimen to 3 days, added low-dose venetoclax (VEN, 200 mg/day) (VCA), and reported the efficacy and safety here. Results: Twenty-five newly diagnosed adult AML patients were enrolled in this study and evaluated for the remission rate after one cycle of the VCA regimen. The CR/Cri was 92%, and all these patients had undetectable minimal residual disease (MRD-). The estimated overall survival at 12 months was 79.3%. The median time for both platelet recovery and absolute neutrophil count recovery was 16 days, faster than that of traditional IC. Compared with the previous CA (4 + 3) regimen, a higher CR rate (92% vs. 80%, P < 0.01) and a deeper degree of remission (CRMRD- rate, 92% vs. 45%, P < 0.01) were found in the VCA group. Conclusions: This study showed that the 3-day CTX and Ara-C regimen is highly effective in newly diagnosed AML patients, and the addition of VEN to the CA regimen achieves higher and deeper one-course remission.

[Clinical Analysis of SET-NUP214 Fusion Gene Positive Patients with Acute Leukemia].

OBJECTIVE: To analyze the characteristics and prognosis of acute leukemia(AL) with SET-NUP214 fusion gene. METHODS: The clinical data of 17 patients over 14 years old newly diagnosed with SET-NUP214 positive AL admitted in Institute of Hematology and Blood Diseases Hospital from August 2017 to May 2021 were analyzed retrospectively. RESULTS: Among the 17 SET-NUP214 positive patients, 13 cases were diagnosed as T-ALL (ETP 3 cases, Pro-T-ALL 6 cases, Pre-T-ALL 3 cases, Medullary-T-ALL 1 case), AML 3 cases (2 cases M5, 1 case M0) and ALAL 1 case. Thirteen patients presented extramedullary infiltration at initial diagnosis. All 17 patients received treatment, and a total of 16 cases achieved complete remission (CR), including 12 cases in patients with T-ALL. The total median OS and RFS time were 23 (3-50) months and 21 (0-48) months, respectively. Eleven patients received allogeneic hematopoietic stem cell transplantation(allo-HSCT), with median OS time of 37.5 (5-50) months and median RFS time of 29.5 (5-48) months. The median OS time of 6 patients in chemotherapy-only group was 10.5 (3-41) months, and median RFS time of 6.5 (3-39) months. The OS and RFS of patients with transplantation group were better than those of chemotherapy-only group (P=0.038). Among the 4 patients who relapsed or refractory after allo-HSCT, the SET-NUP214 fusion gene did not turn negative before transplantation. While, in the group of 7 patients who have not relapsed after allo-HSCT till now, the SET-NUP214 fusion gene expression of 5 patients turned negative before transplantation and other 2 of them were still positive. CONCLUSION: The fusion site of SET-NUP214 fusion gene is relatively fixed in AL patients, often accompanied by extramedullary infiltration. The chemotherapy effect of this disease is poor, and allo-HSCT may improve its prognosis.

Clinical characteristics and risk factors of Aeromonas bloodstream infections in patients with hematological diseases.

BACKGROUND: To analyze the clinical features, risk factors and outcomes of Aeromonas bloodstream infections (BSIs) in patients with hematological diseases to establish an effective optimal therapy against it. METHODS: A retrospective study was performed by reviewing medical records of patients admitted to a tertiary blood disease hospital in China. Patients with hematological diseases who suffered from Aeromonas bacteremia during January 2002 to December 2020 were enrolled in this study. RESULTS: A total of 63 patients who developed Aeromonas bacteremia were enrolled in the study, and 91.9% of patients were neutropenic at the onset of BSIs. The major complications were skin and soft tissue infection (SSTI) (22.2%), followed by gastroenteritis (19.0%) and pneumonia (14.3%). High carbapenem resistance rates (70.8% for imipenem, 71.4% for meropenem) were note among the cases. Furthermore, Aeromonas strains isolated from five individuals developed resistance to quinolone, ß-lactams and tigecycline during the therapy. The 30-day mortality rate was 15.9%, while bacteremia with SSTI showed a much worse prognosis, with 50.0% (7/14) of the patients dying within 30 days of initiating the therapy. In the multivariate analysis, SSTI (OR = 28.72; 95% CI, 1.50-551.30; P = 0.026) and shock (OR = 47.58; 95% CI,1.06-2126.80; P = 0.046) were independent risk factors for mortality. CONCLUSIONS: Aeromonas bacteremia usually occurred in patients with neutropenic status, and patients with SSTIs were more likely to show a worse prognosis. Carbapenems should be avoided in patients with Aeromonas BSIs and SSTIs given high resistance rate.

Combination of cyclophosphamide and cytarabine as induction regimen for newly diagnosed adult acute myeloid leukemia.

Optimizing the induction therapy of acute myeloid leukemia (AML) may improve the remission rate and reduce the risk of relapse, thereby extend survival. Cyclophosphamide (CTX) shows benefit in treating relapsed and refractory AML patients, but it has not been reported in first-line induction regimens. To assess the efficacy and safety of CTX and moderate-dosage cytarabine (Ara-C) as induction chemotherapy in newly diagnosed adult AML, 40 patients were enrolled to receive CTX (20 mg/kg/d) for 4 consecutive days and Ara-C for 3 (1 g/m2 q12h, CA4+3) or 5 (1 g/m2 qd, CA4+5) days. With one course of induction chemotherapy, the overall response rate and the complete remission rate (CR) was 82.5% (33/40) and 77.5% (31/40), respectively. The expected 5 years overall survival and relapse-free survival was 64% in patients experienced CR and fulfilled consolidation therapy. The neutrophil and platelet recovery time were 17 (range, 10-20) days and 16.5 (range, 12-30) days in the CA4+3 group, faster than that of 20 (16-36) days and 20 (14-36) days in the CA4+5 group (P = .006 and P = .006). The cyclophosphamide and cytarabine (CA) regimen was generally safe and had reversible adverse effects. The patients who failed to respond to the CA regimen did not benefit from a second course of other traditional induction chemotherapy either. In conclusion, the combined regimen of CTX and Ara-C represents a promising therapeutic approach to induce the first CR of newly diagnosed adult AML.

Discovery and validation an eight-biomarker serum gene signature for the diagnosis of steroid-induced osteonecrosis of the femoral head.

Steroid-induced osteonecrosis of the femoral head (SONFH) is difficult to be diagnosed at the early stages when it can be administrated effectively. Yet, to date no study has been performed to identify diagnostic biomarkers and to develop diagnostic models for SONFH. In the current study, a total of 60 SONFH patients with Association Research Circulation Osseous (ARCO) stages I-IV, and 20 controls were enrolled and divided into the discovery and validation cohorts. The serum samples were collected and the gene expression profiles were detected by microarray analysis based on the discovery cohort. Then, eight genes (BIRC3, CBL, CCR5, LYN, PAK1, PTEN, RAF1 and TLR4) were identified as the candidate serum biomarkers of SONFH due to the significant differential expression patterns and the topological importance in the interaction network of SONFH-related differentially expressed genes. Functionally, these candidate serum biomarkers were significantly involved into several pathological processes during SONFH progression, such as the immune regulation and inflammation, bone metabolism and angiogenesis. After that, a prediction model for the diagnosis of SONFH was constructed using Partial least squares regression based on the serum levels of the candidate biomarkers. Notably, both the 10-fold cross-validation and the independent dataset test demonstrated the good performance of this model. In conclusion, our study discovered eight promising serum biomarkers and developed the multi-biomarker-based prediction model as a new, potential and non-invasive diagnostic tool for the detection of SONFH, as well as benefit the administration of SONFH in a daily clinical setting.

[Efficacy Comparison of Different Salvage Treatment Regimens for Patients with Refractory/Relapsed Acute Myeloid Leukemia].

OBJECTIVE: To compare the efficacy and safety of 3 different regimens, namely MAC, FLAG and CAG, as the re-induction chemotherapy for acute myeloid leukemia(AML) patients with primary induction failure and relapse. METHODS: The clinical data of 156 AML patients with primary induction failure and relapse, except patients with acute promyelocytic leukemia(APL), treated with any of the above 3 regimens in our center from January 2008 to April 2016 were analyzed retrospectively. According to the treatment regimens, 156 patients were divided into MAC group (n=60), FLAG group (n=45) and CAG group (n=51). The complete remission(CR), partial remissison(PR), overall survival(OS), disease-free survival(DFS) and adverse events during the treatment were analyzed, so as to compare and evaluate the efficacy and safety of the 3 different regimens. RESULTS: After 1 course of re-induction chemotherapy, CR in MAC group was significantly higher than that in FLAG and CAG group (55.4% vs 34.1% vs 34.0%)(P<0.05). The OS was not statistically significantly different among 3 groups (P>0.05) with a median OS of 11 months, 5.46 months and 10.2 months, respectively. The myelosuppression was the main adverse event with no significant difference among the groups(P>0.05). More patients treated with MAC regimen underwent febrile neutropenia (93.3% vs 86.7% vs 64.7%)(P<0.001). However, the incidence of fatal infections was not signicantly different among 3 groups(5% vs 8.9% vs 5.9%)(P>0.05). CONCLUSION: Compared with FLAG and CAG regimen, the MAC regimen can enable more AML patients with primary induction failure and refractory to achieve CR without increasing severe adverse events,therefore,this regimen may provide a opportunity for patients to recieve hematopoietic stem cell transplantation.

[Preliminary Clinical Efficiency of Autologous Peripheral Blood Mononuclear Cells for Treating Critical Limb Ischemia of Thromboangiitis Obliterans].

OBJECTIVE: To evaluate the long-term clinical effect of autologous peripheral blood mononuclear cells (PB-MNC) on critical limb ischemia (CLI) in patients with thromboangiitis obliterans (TAO) patients. METHODS: The clinical data of 22 patients with CLI caused by TAO from July 2004 to May 2013 were analyzed retrospectively, 22 patients were divided into 2 groups; out of them 12 cases in one group were treated with granulocyte colony-stimulating factor (G-CSF)-mobilized autologous peripheral blood mononuclear cells (auto-PBMNC group), 10 cases in another group received conservative treatment (CT group). The log-rank test was used to compare the long-term outcomes in auto-PBMNC group and CT group. RESULTS: The wound healing rate (P=0.016) and CLI-free rate (P=0.013) were significantly higher in PB-MNC group compared with that in CT group. No difference was found in amputation rates between the 2 groups (major amputation: P=0.361, minor and major amputation: P=0.867). No patients died or no serious adverse events occurred during the follow-up period. CONCLUSION: The auto-PBMNC therapy can significantly promote the wound healing, and protect against CLI in TAO patients, but the risk of amputation is not low in comparison with conservative treatment.

Analysis of possible factors relating to prognosis in autologous peripheral blood mononuclear cell transplantation for critical limb ischemia.

BACKGROUND AIMS: Autologous transplantation of granulocyte colony-stimulating factor-mobilized peripheral blood mononuclear cells (M-PBMNCs) has been shown to be effective in treating critical limb ischemia (CLI); however, the studies of the possible prognosis predictors after autologous M-PBMNC transplantation are inadequate. The objective of the study was to assess the possible factors affecting the results of M-PBMNC transplantation for CLI. METHODS: We reviewed the clinical profiles of 87 patients with CLI who were treated with M-PBMNC implantation in the Blood Diseases Hospital, Chinese Academy of Medical Sciences, between December 2002 and December 2011, and we followed these patients. The patients were divided into a good prognosis group and a poor prognosis group on the basis of whether amputation was performed. The significant differences of clinical variables between two groups were analyzed by means of the Mann-Whitney test and χ2 test, and logistic regression analysis was used to study the variables representing the possible prognostic factors for amputation. RESULTS: Of the 87 patients, three patients died and one patient was lost during the follow-up period. We analyzed 83 patients. The diseases included CLI complicated by diabetes mellitus gangrene (35 cases, 42.2%), arteriosclerosis obliterans (31 cases, 37.3%) and thromboangiitis (17 cases, 20.5%). The mean age was 62 years (range, 30-87). The median follow-up time for the surviving patients was 5 years. The 5-year amputation-free rate was 72.2%, and no adverse effects related to M-PBMNC transplantation were observed. CONCLUSIONS: The significant prognostic factors associated with poor angiogenesis were fibrinogen > 4 g/L and fasting blood glucose > 6 mmol/L.

Genetic association of the P-glycoprotein gene ABCB1 polymorphisms with the risk for steroid-induced osteonecrosis of the femoral head in Chinese population.

Steroid administration, which is commonly performed for the treatment of autoimmune inflammatory diseases, cancers or organ transplantation, has been a leading cause of nontraumatic osteonecrosis of the femoral head (ONFH). Single nucleotide polymorphisms (SNPs) of the adenosine triphosphate-binding cassette B1 (ABCB1) gene have been demonstrated to be related to steroid-induced ONFH in small sample sizes of Japanese kidney failure and Chinese systemic lupus erythematosus patients. However, there are obvious controversial results in the relationship of ABCB1 gene polymorphisms with steroid-induced ONFH. The aim of this study was to validate the genetic association of ABCB1 polymorphisms with the risk for steroid-induced ONFH in a large cohort of Chinese population. A case-control study was conducted, which included 94 and 106 unrelated patients after steroid administration recruited from 14 provinces in China, respectively. Two SNPs (rs1045642 and rs2032582) within ABCB1 were genotyped using Sequenom MassARRAY system. Multivariate analyses based on clinical information were performed to determine the associations between the SNPs and risk of steroid-induced ONFH. rs1045642 SNP was significantly associated with steroid-induced ONFH group in codominant (P=0.02), recessive (P=0.006) and overdominant (P=0.03) models. However, there were no differences found in genotype frequencies of rs2032582 SNP between controls and patients with steroid-induced ONFH (all P>0.05). These findings suggested that rs1045642 SNP of ABCB1 may be associated with the risk of steroid-induced ONFH. Thus, it is useful to analyze this polymorphism for identifying high-risk individuals before the administration of steroids.

Association of a polymorphism in PON-1 gene with steroid-induced osteonecrosis of femoral head in Chinese Han population.

BACKGROUND: Treatment with steroids covers a wide spectrum of diseases in clinic. However, some users are suffering from serious side effects of steroid administration, while we enjoy the benefit it brings about. Osteonecrosis of the femoral head (ONFH) is a troublesome one among them. Recent studies have demonstrated that lipid metabolism disorder may play a vital role in pathogenesis of ONFH and mutation of the paraoxonase-1 (PON-1) gene may be involved in the occurrence of this disease. However, the relationship between polymorphisms of PON-1 and ONFH has not been thoroughly studied. The aim of this study was to determine whether PON-1 polymorphisms are associated with steroid-induced ONFH through a cohort study among Chinese Han population. METHODS: This trial applied a case-control scheme to compare the clinical data including PON-1 SNP among 94 patients and 106 control subjects to analyze the association between SNP and risk of steroid-induced ONFH. Time of Flight Mass Spectrometer is utilized for genotyping and the result was analyzed in multivariate analysis models. RESULTS: According to polymorphism test of rs662, its SNP was significantly associated with the risk of ONFH in overdominant analysis model [P value: 0.022; odds ratio (OR): 0.39]. However, genotype frequencies of rs662 of PON-1 gene between case and control group showed no differences (P > 0.05). CONCLUSIONS: Our data suggest for the first time that SNP (rs662) of the PON-1 gene was associated with the risk of steroid-induced ONFH. In addition, PAI-1 SNPs may play an important role in pathogenesis of ONFH. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticphatology.diagnomx.eu/vs/1501829501107336.

Genetic polymorphisms in plasminogen activator inhibitor-1 predict susceptibility to steroid-induced osteonecrosis of the femoral head in Chinese population.

BACKGROUND: Steroid usage has been considered as a leading cause of non-traumatic osteonecrosis of the femoral head (ONFH), which is involved in hypo-fibrinolysis and blood supply interruption. Genetic polymorphisms in plasminogen activator inhibitor-1 (PAI-1) have been demonstrated to be associated with ONFH risk in several populations. However, this relationship has not been established in Chinese population. The aim of this study was to investigate the association of PAI-1 gene polymorphisms with steroid-induced ONFH in a large cohort of Chinese population. METHODS: A case-control study was conducted, which included 94 and 106 unrelated patients after steroid administration recruited from 14 provinces in China, respectively. Two SNPs (rs11178 and rs2227631) within PAI-1 were genotyped using Sequenom MassARRAY system. RESULTS: rs2227631 SNP was significantly associated with steroid-induced ONFH group in codominant (P = 0.04) and recessive (P = 0.02) models. However, there were no differences found in genotype frequencies of rs11178 SNP between controls and patients with steroid-induced ONFH (all P > 0.05). CONCLUSIONS: Our data offer the convincing evidence for the first time that rs2227631 SNP of PAI-1 may be associated with the risk of steroid-induced ONFH, suggesting that the genetic variations of this gene may play an important role in the disease development. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1569909986109783.

[Organ toxicity and efficacy of high-dose daunorubicin-based chemotherapy in the treatment of acute leukemia].

OBJECTIVE: To evaluate the safety and therapeutic effect of high-dose daunorubicin-based (HD-DNR) chemotherapy in the treatment of acute leukemia (AL). METHODS: The clinical data of 25 AL patients, including 14 cases for induction chemotherapy, 8 for consolidation chemotherapy and 3 for reinduction therapy, which were treated with HD- DNR (DNR dosage of 90 mg/m(2)× 3 d) between June 2010 and August 2012 in our hospital were retrospectively analyzed, the adverse reaction of chemotherapy, especially cardiac toxicity and therapeutic effect were evaluated. RESULTS: Most of the adverse reactions were mild, including cardiac toxicity, and no patient discontinued therapy because of HD-DNR related toxicities. Grade 3 or higher adverse reactions occurred only in the infection (56%) and diarrhea (12%). Withdrawal or dose reduction due to strong adverse reactions was not observed in all patients. Adverse reactions of infections (92%), lower ejection fraction(52.6%), diarrhea (48%), nausea (36%), vomiting (36%), dental ulcer (36%) and myocardial ischemia (32%) were relatively more common. The median time of neutrophil count reached to ≥ 0.5 × 10(9)/L and platelet ≥ 20 × 10(9)/L were both 21 days(ranged 9-31 and 9-38 days). Nine patients were complicated with infections before chemotherapy and 14 after chemotherapy, mainly occurred in gastrointestinal tract and respiratory system. Gastrointestinal, liver and kidney toxicity was slight. The cardiac ejection decreased in 10 cases, but only 1 reached grade 2 without clinical symptoms. Of the 14 AL patients for induction chemotherapy, 13 achieved hematological complete remission. Eight patients received HD-DNR as consolidation chemotherapy remained complete remission, while 3 refractory/relapsed patients remained non-remission. CONCLUSION: The adverse reaction of HD-DNR based chemotherapy for AL treatment was mild, no obvious cardiac adverse reaction occurred. The treatment dose of DNR at 90 mg/m(2) × 3 d can be safely and effectively used to treat acute leukemia.

[Method of expansion of late endothelial progenitor cells].

OBJECTIVE: To validate a novel method of expanding late endothelial progenitor cells (EPC) in vitro. METHODS: We cultured mononuclear cells (MNC) from human peripheral blood on the plate with the feeder layer cells, i.e. irradiated late EPC or human umbilical vein endothelial cells. After 21 days, the numbers of late EPC colonies were counted separately. And the surface antigen of late EPC was detected by fluorescence-activated cell sorter (FACS) and their in vitro ability of forming vascular structure examined by matrigel. RESULTS: Both colony numbers of late EPC with feeder layer cell culturing were over 20 times than those without (40.0 ± 3.9, 39.3 ± 3.1 vs 2.0 ± 1.3, both P < 0.05). And the former's late EPC colony appeared earlier. The late EPC expressed CD31, CD34, eNOS, Flt-1, P1H12, Sendo, VE cadherin and CD117. And vascular structures were discerned. CONCLUSIONS: The method of feeder layer cells may vastly expand the quantity of late EPC. And microenvironment plays an important role in its expansion.

Role of microenvironment in the process of expansion of late endothelial progenitor cell in vitro.

OBJECTIVE: To study the role of the feeder layer cells as niche in the process of expansion of late endothelial progenitor cell in vitro. METHODS: We cultured mononuclear cells (MNC)from human peripheral blood (PB)on the plate with the feeder layer cells which were irradiated late endothelial progenitor cells(EPC)or human umbilical vein endothelial cells (HUVEC) by EGM-2. After 21 days, the numbers of obtained late EPC colonies were counted separately, and their surface antigen of the late EPC was verified by fluorescence-activated cell sorter (FACS) analysis, and their ability of forming vessel structure with Matrigel in vitro. The differentiation of single stem cell on the feeder layer cell was traced by video-microscopy. RESULTS: After 21 days of culture,(40.0±3.9)and(39.3±3.1)late EPC colonies that MNC of a hundred milliliter PB were cultured, respectively, on the feeder layer cells of EPC and HUVEC were much more than (2.0±1.3) colonies cultured on without the feeder layer cells (all P <0.05). These cells also expressed CD31,CD34,eNOS,FLt-1,P1H12,Sendo,VE cadherin,and CD117, as shown by FACS analysis. Furthermore, they formed vessel structure with Matrigel in vitro. The video-microscopy showed the asymmetric cell division was participated by the feeder layer cell during the expansion of single stem cell. CONCLUSION: The massive expansion of late EPC can be achieved by the provision of the feeder layer cells, which may be involved in the stem cell asymmetric cell division.

[Analysis the advantage of autologous mobilized peripheral blood mononuclear cells transplantation on lower limbs ischemia disease].

OBJECTIVE: To analyze the efficacy and its correlation with species of transplant cells of autologous mobilized peripheral blood (PB) mononucleated cells (MNCs) transplantation on 59 patients with lower limbs ischemia. METHODS: Fifty-nine patients were evaluated with symptoms scores and after that their PBMNCs were mobilized and collected and then injected into the ischemic area at equal distance. They effectiveness and scores were evaluated at 7th day and 4th month after therapy. The correlation of CD34(+) cells and of MNCs with effectiveness was analysed respectively, and formula for correlations between them and effectiveness was calculated. RESULTS: After MNCs injection, the effectiveness was observed both at 7th day and 4th month. The correlation of MNCs with effectiveness was stronger than that of CD34(+) cells (the effectiveness was represented by nimodipine value), According to the formula of nimodipine value, the value of the latter = 0.484 + 1.055 × CD34(+) cells number and the former = 0.288 + 0.401 × MNCs number with a correlation coefficient of R = 0.461 (P = 0.047) and R = 0.473 (P = 0.000) respectively. CONCLUSION: Autologous mobilized PBMNCs number is a better indicator for effectiveness than CD34(+) cells number.

Thrombocytopenia in patients with systemic lupus erythematosus: significant in the clinical implication and prognosis.

The purpose of this investigation was to determine the characteristics of the clinical manifestations, laboratory findings and prognostic impact of the thrombocytopenia patients with systemic lupus erythematosus (SLE). The case group of 47 SLE patients with thrombocytopenia and the control of 49 SLE patients with normal platelet number were reviewed retrospectively. The clinical manifestations, immunological profiles, hemograms and myelograms, disease activity (SLEDAI-2K), end organ damages (SLICC) and survival rate at the time of follow-up were recorded. The analysis of clinical manifestations showed that the case group was more likely to be with renal disease (p = 0.038), and less skin rash (p = 0.032). The analysis of the hemograms revealed that the case group was more concomitant with anemia, leucopenia and neutropenia than the control group (p < 0.001, p = 0.014 and p = 0.02, respectively). Moreover, it was also revealed that the SLE patients with thrombocytopenia were often in the condition of higher disease activity, had more probability of end organ damage, and were associated with a higher mortality rate (P = 0.041, p = 0.035 and p = 0.038, respectively). The clinical data and laboratory findings were different between the SLE patients with thrombocytopenia and those with normal platelet number. Thrombocytopenia can be recognized as a reliable prognostic marker to identify a subset of patients with higher disease activity, more possibility of end organ damage and higher mortality.

[Analysis of complicated anemia in 60 patients with systemic lupus erythematosus].

The study was aimed to explore the characteristics of clinical manifestation, laboratory indicators and bone marrow examination of SLE patients with anemia. 60 SLE patients with anemia were analyzed for their clinical manifestation, laboratory indicators and bone marrow examination in comparison with 40 contemporaneous SLE patients without anemia. The results indicated that there were significant differences in clinical manifestations of fatigue between the SLE patients with anemia and those without anemia. The detection rate of the decreased Plt and C4 and the percentages of eosinophils, early normoblast, polychromatic normoblast and orthochromatic normoblast in bone marrow were all higher than that in those without anemia. The ANA with titer 1:320 in SLE patients complicated by anemia was lower than that in those without anemia. In conclusion, the clinical manifestation, experimental examination and bone marrow findings were significantly different between the SLE patients with anemia and without anemia.

[Clinical analysis of 438 patients with essential thrombocythemia].

OBJECTIVE: To analyse the clinical feature and natural course of essential thrombocythemia (ET). METHODS: A retrospective analysis was conducted in ET patients treated in our hospital during May 1980 to December 2006. RESULTS: Four hundred and thirty eight patients (201 males and 237 females with a median age of 48 years) were diagnosed. Hemorrhage occurred in 101 cases (23.1%), thrombosis in 86 cases (19.6%), and both hemorrhage and thrombosis in 13 cases (3.0%). Splenomegaly occurred in 150 cases and hepatomegaly occurred in 60 cases. One hundred and forty-nine cases (34%) had no symptoms at diagnosis and 145 cases (33.1%) confirmed by routine blood tests due to other diseases. The median platelet count at diagnosis was 1000 x 10(9)/L [(533 -3740) x 10(9)/L]. Bone marrow biopsy was performed in 255 cases which showed mainly increase of enlarged mature megakaryocytes with hyper-lobulated nuclei and local proliferation of reticular fiber was revealed in 51 cases. JAK2V617F mutation was detected in 90(78.9%) of 114 patients studied. Karyotype analysis was performed in 180 cases and 6 (3.3%) had clonal chromosomal aberrations. Two hundred and sixty-one patients were followed up over 12 months with a median of 60 months (range from 12 to 300 months). Seventeen cases (6.5%) evolved into marrow fibrosis (MF) and one case into polycythemia vera (PV). One case evolved into PV 6 years and then MF 20 years after diagnosis of ET. Three cases developed acute monocyte leukemia (M5), myelodysplastic syndrome (MDS) and multiple myeloma (MM), respectively. CONCLUSIONS: ET is a chronic myeloproliferative disorder characterized predominantly by thrombocytosis and hemorrhage. The percentage of asymptomatic cases is high. The prognoses for most cases were good with a few cases may evolve into MF.