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  • NLM标题: Indian J Hematol Blood Transfus
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  • 机译 低蛋白Z水平:急性冠脉综合征的血栓形成风险生物标志物。
    摘要:
  • 机译 类风湿关节炎患者肿瘤坏死因子抑制剂治疗后获得的VIII因子抑制剂
    摘要:
  • 机译 通过RhD阴性献血者中的混合恒河猴盒分子分析进行RHD基因分型
    摘要:D antigen is the most important and immunogenic antigen of the Rh blood group. The RhD-negative phenotype has different genetic backgrounds with variable distribution in different populations. Hybrid Rhesus box, resulting from RHD gene deletion, is used in genotyping studies of the Rh blood group as a marker to identify the RHD gene deletion. This study for the first time identified genetic mechanisms for the occurrence of RhD-negative phenotype among the Iranian population. 200 RhD-negative blood donors were randomly selected from Tehran Blood Transfusion Center. The phenotype of D, C, Ε, e and c antigens was serologically identified, and DNA was extracted from buffy coat. The molecular analysis of hybrid Rhesus box was performed by PCR-SSP and PCR-RFLP. Moreover, the presence of different exons of RHD gene was investigated by real-time PCR on extracted DNA. Hybrid Rhesus box was detected in all samples, and PCR-RFLP confirmed that 198 (99%) were homozygous for an RHD gene deletion and 2 were heterozygous for hybrid Rhesus box in which one (0.5%) had a weak D type 11 and the other one (0.5%) had a RHD-CE (2-9)-D2 hybrid allele. Similar to Caucasians, the frequency of RHD gene deletion was high among the Iranian population studied in this investigation, so hybrid Rhesus box can be used as an efficient marker to detect RHD gene deletion in our population.
  • 机译 Haematocon 2018高知
    • 作者:
    • 刊名:Indian Journal of Hematology Blood Transfusion
    • 2018年第Suppl 1期
    摘要:
  • 机译 靶向治疗黎明在印度的淋巴瘤
    摘要:
  • 机译 遗传性大血小板减少症:相关的形态学,流行病学,分子病理学和临床特征
    摘要:Inherited macrothrombocytopenia is increasingly being recognized as a relatively common condition. This descriptive review aims at focusing on the different areas of advancement that have taken place with this condition with particular reference to India. A pubmed search of articles between January 1990 and October 2017 with the key words-macrothrombocytopenia, asymptomatic macrothrombocytopenia, macrothrombocytopenia India, syndromic macrothrombocytopenia, molecular pathology, megakaryopoiesis and platelet formation were searched. The shortlisted articles were then read. Review articles provided additional references and the articles thus obtained were also read. Special interest and research conducted by the authors provided further sources of information. A total of 487 articles were found of which 68 articles were related to our subject of review. Review articles were read and additional articles from the reference quoted. Forty-four percent of nonsyndromic Inherited macrothrombocytopenia showed mutations of MYH9, GP1BB, GP1Ba, GPIX, ABCG5 and 8, ACTN, FLI, TUBB and RUNX1 frequently in heterozygous state. All types of inheritance pattern namely autosomal dominant, recessive and sex linked patterns have been described. Syndromic causes of this phenomenon are well known and have been described. Many asymptomatic patients do have mild or moderate bleeding history. Clinical algorithms to differentiate chronic ITP associated macrothrombocytopenia from inherited variety have been explored. Inherited macrothrombocytopenia is an emerging area of interest in platelet biology with its implication in diagnosis, prognosis, genetic counseling, management and in transfusion medicine.
  • 机译 强化“小儿”型方案治疗的年轻成人急性淋巴细胞白血病
    摘要:Young adults with acute lymphoblastic leukemia do better when treated on “pediatric” protocols. Young adults (18–30 years) with Ph-negative ALL treated between 2000 and 2014 were retrospectively analyzed. Two-hundred and thirty-two patients were included [median age 21 years (18–30); 176 (76%) males; median WBC 16,000/cmm]. Protocols used were: BFM 95 (N = 147, 63%), MCP-841 (N = 51, 22%), GMALL (N = 21, 9%), INCTR (N = 9, 4%) and UKALL (N = 4, 2%). Complete remission was achieved in 194/232 (84%). Twenty patients (9%) died due to toxicity which was higher with BFM versus others (18/147 vs. 2/85; p = 0.031). After a median follow-up of 48 months, median RFS and OS were 35.5 months (25–46), and 25 months (18–31) and actuarial RFS and OS (5-years) were 45% (37–53) and 39% (32–46). BFM protocol improved RFS (51 vs. 35%, p = 0.027) but not OS (43 vs. 33%, p = 0.2). The survival outcomes reported are 15–20% lower than those reported from West. Better supportive care and risk-adapted therapy may improve outcomes.Electronic supplementary materialThe online version of this article (doi:10.1007/s12288-017-0892-y) contains supplementary material, which is available to authorized users.
  • 机译 急性白血病患者的临床资料和结核病结果分析
    摘要:Patients with acute leukemia (AL) are predisposed to develop infections including tuberculosis (TB). The risk is specifically higher in patients from TB endemic areas. Patients (≥12 years) with AL treated between January-2014 to January-2017 who developed TB were reviewed. Patients were classified into three groups: acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML) and acute promyelocytic leukemia (APML) and a systematic analysis of clinical features and outcomes was conducted. Over the study period, 26 patients of AL developed TB. The median time to diagnosis of TB was 8 weeks (0–432 weeks) following the diagnosis of AL and it was comparable between the three leukemia groups. The diagnosis of TB required alteration of anti-leukemia therapy in 26.9% patients and rescheduling in another 42.3% patients. Therapy alteration/rescheduling were more frequent in patients with AML as compared to ALL and APML (p < 0.03, <0.04). Disseminated TB was more common in AML patients (p < 0.016). ATT could be successfully administered in 86.9% patients with improvement of TB. The incidence of ATT induced hepatitis was 34.9%. Mortality was directly attributable to TB in 10% patients. Managing tuberculosis remains a challenge during treatment of acute leukemia. With this analysis, we advocate for a need of early suspicion and evaluation for TB in patients receiving treatment for acute leukemia. Rescheduling and or alteration of anticancer therapy due to TB is associated with significantly higher mortality. Therefore, in carefully selected cases, antileukemia therapy should continue after starting ATT as early as possible.
  • 机译 在印度患者中使用尼洛替尼的经验
    摘要:Important genetic and ethnic factors could affect the toxicity and efficacy of tyrosine kinase inhibitors. Though nilotinib has been available in India since 2010, there is no report on its safety and toxicity from Indian patients with chronic myeloid leukemia. This is an analysis of efficacy and toxicity of nilotinib when used as a second line drug after failure or intolerance to imatinib. Thirty-seven patients started nilotinib [median age 46 years, median duration from diagnosis 5 years, 73% in chronic phase at time of switch] between 2010 to 2016. Reason for switch: failure of imatinib in 33 (89%) and intolerance in 4 (11%). Starting dose 600 mg/day. Dose modifications: 15 (40%) patients required initial dose modifications, but subsequently 25 (67%) patients could tolerate 600 mg/day. Nine (24%) patients were able to tolerate 800 mg/day. The commonest grade 3/4 toxicities were thrombocytopenia (n = 9, 24%), hyperbilirubinemia (n = 7, 18%) and leukopenia (n = 3, 8%). Six patients (16%) discontinued nilotinib due to toxicity while 8 (21%) stopped due to lack of efficacy. After a median duration of 14 months among those continuing nilotinib, 54% of patients responded which included 14 patients who achieved CHR and seven who achieved major molecular response. In the first report on use of nilotinib in Indian patients, we observed a higher incidence of liver toxicity compared to previous reports. This should be seen the context that all these patients received nilotinib as second line therapy.
  • 机译 印度十字路口的骨髓瘤
    摘要:
  • 机译 植入综合征:自体干细胞移植的临床特征和预测因素。
    摘要:Engraftment Syndrome (ES) maybe observed in patients who undergo autologous stem cell transplant (SCT). To investigate clinical criteria for ES diagnosis and analyse the risk factors for this complication, we reviewed all auto-SCT cases (Lymphoma and Myeloma) performed during the past 9 years at two tertiary care centres. We analysed all patients with a non-infectious fever, developed within 7 days of engraftment (first day of ANC of 500 on two consecutive days) in 178 patients undergoing autologous stem cell transplant. A total of 46/178 (25.8%) patients developed non-infectious fever and one or more clinical signs of ES within 7 days of engraftment. In all, 29 (61%) fulfilled the Maiolino and 12 (26%) the Spitzer criteria. The incidence of engraftment syndrome using the Maiolino criteria in our study was 29 (15%), which compares well with Spanish study (13% using same criteria) and the original Maiolino study (20%). All patients with ES satisfactorily recovered and discharged with a median of 20 days from hospital. There was no significant difference in number of days of hospitalisation and days of antibiotics between the ES and non ES arms. All patients recovered without any morbidity and only 1 (2%) patient required readmission for fungal pneumonitis. 8 (17%) patients required ICU admission due to delay in initiation of steroids. None of the factors including number of chemotherapy cycles, conditioning regime, disease status, CD34 collection, growth factors and day of WBC engraftment except female (p = 0.064) were statistically significant (in univariate or multivariate analysis). Our study shows that engraftment syndrome is common in autologous transplant setting. Maiolino criteria to diagnose ES is more sensitive in our setting. If detected and treated early there is not much morbidity or mortality related to ES.
  • 机译 保持P阳性的PACE对B谱系急性淋巴细胞白血病中的类似ph值的检测:资源受限的环境中的一种实用且具有成本效益(PACE)的方法
    摘要:Philadelphia (Ph)-like or BCR-ABL like acute lymphoblastic leukemia (ALL) is defined on the basis of a gene expression profile that is similar to Ph-positive ALL. It comprises a wide spectrum of genetic lesions affecting primarily the cytokine receptor and/or kinase signalling genes. It accounts for approximately 10–15% of pediatric ALL, and is more common in patients who are high-risk according to the National Cancer Institute criteria. Presence of Ph-like mutations is an independent predictor of poor outcome. However, there is vast potential to utilize targeted therapy to improve survival in this group. The sizeable range of genetic lesions varying from translocations, fusions, point mutations and deletions make the diagnosis challenging. Hence, a practical and cost effective approach is required to enable identification in resource constrained settings. Patients with recurrent cytogenetic abnormalities such as ETV6-RUNX1, high hyperdiploidy, TCF3-PBX1, BCR-ABL1 and KMT2A (MLL) rearrangement need not be tested, as these are mutually exclusive with BCR-ABL like mutations. Detection of CRLF2 overexpression, which is the commonest abnormality, is employed as the first step. In patients lacking overexpression, testing for tyrosine kinase fusions can be performed. However, the goal should be to employ a combination of molecular diagnostic techniques such as reverse transcriptase polymerase chain reaction (PCR), real time quantitative PCR, fluorescence in situ hybridization and Sanger sequencing to detect genetic lesions that are amenable to targeted therapy.
  • 机译 侵略性CD 20阳性B细胞淋巴瘤中的DA-EPOCH-R:现实世界的经验
    摘要:Recent reports have shown that excellent survival outcomes can be achieved in adult Burkitt’s lymphoma with the use of DA-EPOCH-R regimen. When compared to earlier intense pediatric-type protocols, this regimen is less toxic. There are limited reports available on the use of this regimen outside the context of clinical trials. We analyzed the outcomes of patients who were treated with the DA-EPOCH-R regimen [Burkitt’s lymphoma (BL), primary mediastinal B cell lymphoma (PMBCL) and HIV-positive patients with diffuse large B cell lymphoma (DLBCL)] at our center over a 3 year period. Baseline characters, responses, and toxicity data was captured from records. Event-free survival (EFS—from therapy initiation till occurrence of event (non-achievement of complete response or relapse) and overall survival (OS—from therapy initiation till death due to any cause) were estimated using Kaplan–Meier method. Among 41 patients [median age 40 years (18–76)], the following diagnoses were included-HIV negative patients (N = 29): BL (N = 24), PMBCL (N = 5); HIV positive patients (N = 12): BL (N = 8), and DLBCL (N = 4). Among those with BL, majority had stage III/IV disease (N = 21/32, 65%). At the completion of planned therapy, 33 had achieved CR (81%). One patient died due to toxicity. The actuarial EFS and OS at 2 years were 80 and 77% respectively for all patients. The OS at 2 years was 100% for PMBCL, 80% for BL and 50% for HIV-positive DLBCL. Majority of the failures in BL were in patients with advanced disease. DA-EPOCH-R can be used in real-world setting and allows treatment of older patients with BL.
  • 机译 骨髓增生异常综合症和急性髓性白血病中染色体7异常的患病率:单中心研究和简要文献综述
    摘要:Chromosome 7 abnormalities in patients with myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) heralds a poor prognosis. However its prevalence, morphological characteristics and clinical impact in MDS and AML in Indian subcontinent is sparsely reported. This was an observational cross-sectional study performed to evaluate the clinico-pathological profiles of MDS/AML patients with chromosome 7 abnormalities over a period of 4 years. 724 cases of MDS (n = 150) and AML (n = 574) were evaluated. Abnormal karyotype was detected in 49% (43/88) patients of MDS and 44% (127/289) cases of AML. Chromosome 7 abnormalities were detected in 18% cases of MDS (16/88) and 6.5% (19/289) cases of AML. Sole chromosome 7 abnormalities were detected in 5.7% (5/88) and 2.7% (8/289) and in adjunct to complex abnormalities in 7.9 and 3.1% cases of MDS and AML respectively. Morphologically, dyserythropoiesis, dysmyelopoiesis and eosinophilia were seen in 100, 66 and 56% cases of MDS and 38, 40 and 21% cases of AML. Majority of the patients had an aggressive natural course and outcome was dismal. Chromosome 7 abnormalities are strongly associated with the presence of morphological dysplasia and eosinophilia, irrespective of the type of aberration. It is invariably associated with very poor outcome.
  • 机译 泊沙康唑预防性诱导化疗在急性髓性白血病患者中的疗效:资源有限环境下的一项观察性研究
    摘要:Large randomized controlled trials have shown significant decrease in incidence of invasive fungal infection in acute myeloid leukemia patients with Posaconazole prophylaxis. However, very less is known about value of Posaconazole prophylaxis in resource limited settings. This observational cohort study evaluated the incidence of fungal infection in patients with hematological malignancies undergoing induction chemotherapy with Posaconazole as antifungal prophylaxis and was compared with historical controls who received Fluconazole as fungal prophylaxis. The study was conducted from Oct 2013 to July 2015 in Department of Hematology of a tertiary care center. Fifty-three patients of acute myeloid leukemia on Posaconazole as fungal prophylaxis and 53 historical controls on Fluconazole as fungal prophylaxis were included for final analysis. Baseline characteristics were well matched between groups. Patients on Fluconazole were more likely to experience breakthrough IFDs (28.3 and 11.3%; p = 0.028) than in patients receiving Posaconazole prophylaxis. No significant difference was observed in overall, attributable mortality or in shift to first line antifungal. Both Posaconazole and Fluconazole were well tolerated with no major adverse effects requiring discontinuation of the drug. Minor side effects were seen in 39 and 47% patients in study and control group respectively. Vomiting and nausea were the commonest side effects seen in both study and control group (26 vs. 34% and 38 vs. 40% of patients, respectively). The results of our study in patients with acute myeloid leukemia provide evidence that Posaconazole prophylaxis significantly decreases the incidence of fungal infection and is well tolerated.
  • 机译 急性粒细胞性白血病患者家庭照顾者中照顾的积极方面的相关性
    摘要:To evaluate the positive aspects of caregiving and its sociodemographic and clinical correlates in family caregivers of patients with Acute Myeloblastic Leukaemia (AML). A total of 30 caregivers of the patients with AML were evaluated on the Scale for Positive Aspects of Caregiving Experience (SPACE), Family Burden Interview (FBI) and Schedule and Caregiver Strain Index (CSI). On the SPACE, the mean score was highest for the domain of motivation for the caregiving role, followed by the domain of ‘caregiving personal gains’, ‘caregiver satisfaction’ and least score was seen in the domain of ‘self-esteem and social aspect of caring’. All the SPACE domains were negatively correlated with financial burden, disruption of routine family activities, disruption of family interaction, physical health and subjective burden domains of FBI. Effect on physical health of others domain of FBI correlated negatively with all domains of SPACE except ‘self esteem and social aspect of caregiving’. FBI total objective burden was negatively correlated with motivation for caregiving role, caregiver satisfaction and total SPACE score. All SPACE domains were negatively correlated with adaptation and inconvenience domains and total CSI score. Lower level of positive caregiving experience is reported by caregivers who experience high level of caregiver burden.
  • 机译 HbE-β-地中海贫血的产前诊断:印度西部一个中心的经验
    摘要:The clinical presentation of HbE-β-thalassemia is extremely variable, however, many cases are severe and transfusion dependent. We offered prenatal diagnosis to 108 couples, 20 of whom came prospectively. CVS was done in 93 cases (9.5–13 weeks of gestation) while amniocentesis/cordocentesis was done for 15 cases in the second trimester. Diagnosis was done by reverse dot blot hybridization, ARMS, DNA sequencing and in a few cases by HPLC analysis of fetal blood. The genetic combinations in the couples at-risk were the following: HbE trait/β-thal trait-95, HbE-thal/HbE trait-5, HbE homozygous/β-thal trait-3, HbE-thal/β-thal trait-3, HbE Lepore/β-thal trait-1, HbE trait/HbDPunjab trait-1. IVS1-5(G>C) was the commonest β-thalassemia mutation followed by codon15(G>A), codon30(G>C), codons41/42(-CTTT), the 619 bp deletion and codon8/9(+G) in the β-thalassemic parent. However, several rare mutations seen in India like -90(C>T), -88(C>T),codon15(-T), IVS1-129(A>C), IVS1-130(G>C), IVSII-1(G>A), IVSII-837(C>T) and IVSII 848(C>A) were also encountered. Twenty-one fetuses were affected (HbE-β-thal-20, β-thal major-1) and all the couples opted for termination of the pregnancies. Couples with affected children wish to undergo prenatal testing for HbE-β-thal in subsequent pregnancies. More regional centers are needed for these services, particularly in West Bengal and the North-East where HbE is very common.
  • 机译 单剂量rasburicicase(1.5 mg)预防和管理实验室肿瘤溶解综合征的功效
    摘要:Rasburicase is a recombinant urate oxidase enzyme approved for use in tumor lysis syndrome (TLS) and it acts by reducing serum uric acid levels. Using rasburicase at the recommended dose of 0.2 mg/kg/day for 5 days is expensive and it is not known whether this extended schedule is clinically beneficial compared to a single fixed dose of 1.5 mg. The aim of the present study was to evaluate the efficacy of single dose rasburicase 1.5 mg in prevention and management of TLS. Rasburicase is available as single use 1.5 mg vial. At our institution a single dose of rasburicase 1.5 mg irrespective of bodyweight has been used in adults and in children a dose of 0.15 mg/kg (maximum 1.5 mg) has been used since 2012 for prevention and management of TLS and subsequent doses are given based on biochemical response and clinical condition. We retrospectively analysed the case records of patients who had received rasburicase from January 2012 to January 2017. The study included 186 patients with hematological malignancies who received rasburicase. Children accounted for 56.4% (n = 105) patients and males comprised 73% (n = 135). Rasburicase was used prophylactically in 59 (31.7%) patients, for laboratory TLS in 76 patients (40.8%) and for clinical TLS in 51 (27.4%) patients. Single fixed dose rasburicase prevented laboratory/clinical TLS in 87% of the prophylactic group and prevented clinical TLS in 72% of the laboratory TLS group. None of the patients in prophylactic and laboratory TLS group developed clinical TLS. However, majority of the patients with clinical TLS required more than one dose rasburicase. Single dose of 1.5 mg (1 vial) rasburicase is efficient in preventing and managing laboratory TLS and is economically viable in resource constrained settings.
  • 机译 小儿镰状细胞病患者的维生素D缺乏症及其与炎症标志物,血脂和调节性T细胞的关系
    摘要:We investigated vitamin D deficiency in pediatric sickle cell disease patients and its association with selected bone, lipid and inflammatory parameters. The study included 64 patients (33 SS and 31 SB) and 21 carriers (AS). Blood was obtained to assess levels of vitamin D, WBC, CRP, Ca, P, ALP, PTH, triglyceride, total cholesterol, LDL, VLDL, HDL, IL-2, IL-12, TNF-α, IL-4, IL-6, IL-10 and regulatory T cells. The patients were grouped according to their genotype (SS, SB) and vitamin D status (low or normal). Carriers were also grouped as low or normal vitamin D. Laboratory findings were similar between low and normal Vit D groups in SS, SB and AS genotypes except a lower IL-12 in SB-low vitamin D compared SB-normal vitamin D group. Acute chest syndrome was more frequent in SS-low Vit D (63%) compared to SS-normal Vit D (25%), SB-low Vit D (21%) and SB-normal Vit D (33%) (P = 0.045). Both SS and SB with low vitamin D had higher VLDL (P = 0.006 and P = 0.022), TNF-α (P = 0.001) and regulatory T cells (P = 0.000) compared to AS-low vitamin D. Both SS and SB with normal vitamin D had higher levels of regulatory T cells (P = 0.000) compared to AS-normal vitamin D. Vit D was not a modifier of selected inflammation, bone and lipid parameters in sickle cell disease. Acute chest syndrome was comparably more frequent in SS-low vitamin D. Increase of regulatory T cells in the patients was a result of chronic inflammation in sickle cell disease.
  • 机译 基于血液分析仪的克隆对非克隆性淋巴细胞增多的白细胞计数数据:真实的测试经验
    摘要:Automated blood counts revealing lymphocytosis necessitate smear reviews. Even expert morphological evaluation may however, fail to differentiate a benign-versus-malignant etiology without further testing. Automated analyser-derived quantitative data on leukocyte cell populations remain undertested for distinguishing such etiologies. Instrument manufacturers claim that if successful, they may be used to generate software flags that help under-resourced laboratories better triage hemogram specimens requiring further testing. We tested the diagnostic accuracy of volume-conductivity-scatter (VCS) indices together with complete blood count (CBC) parameters in such scenarios. We compared LH780-derived (Beckman Coulter, FL, USA) CBC + VCS parameters from patients with clonal lymphoproliferations (n = 42, including 30 chronic lymphocytic leukemia cases) versus 83 controls with absolute or relative lymphocytosis (derivation cohort). Diagnostic performances of 11 logistic regression equations derived were subsequently evaluated on two specific validation cohorts (n = 130 and n = 1465). Clonal lymphocytoses showed significantly lower hemoglobin and higher leukocyte counts but similar lymphocyte percentages (LY %) vis-à-vis controls. The most significant, albeit overlapping predictor of clonality was the absolute lymphocyte count, LY# (47.8 ± 48.4 × 109/L vs. 2.9 ± 1.4 × 109/L in clonal vs. benign cases). In eleven logistic regression equations constructed using four combinatorial approaches, only the models with LY# (highest sensitivity/specificity of 99.3%/100%) and the lymphocytic VCS parameters alone (highest sensitivity/specificity of 76.2%/90.2%) performed consistently in both validation cohorts. Lymphocytic VCS parameters were moderately successful in distinguishing benign-versus-malignant lymphocytes. Other approaches of CBC-plus-VCS parameters did not sustain their initial excellent performances in the validation cohorts, highlighting a need for careful appraisal and better standardization of automated cellular analysis technologies.

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