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Evaluation of serum level of lymphoid enhancer-binding factor-1 and its relation with clinico-hematological and prognostic parameters in pediatric patients with acute lymphoblastic leukemia 急性淋巴细胞白血病患儿血清淋巴增强因子结合因子-1水平及其与临床血液学和预后参数的关系
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_1_22
Zeena Ahmed, A. Ahmed
BACKGROUND: Acute lymphoblastic leukemia (ALL) is a heterogeneous disorder characterized by the proliferation of immature lymphoid cells that accumulate in the bone marrow, peripheral blood, and extramedullary sites, causing the clinical manifestations of the disease. Lymphoid enhancer-binding factor-1 (LEF1) is a target gene and central mediator for the Wingless-type signaling pathway, and it has an important role in normal hematopoiesis. High LEF1 expression was reported as a prognostic marker in many types of hematological and nonhematological malignancies. AIM OF THE STUDY: To evaluate the serum level of LEF1 in pediatric patients with ALL and its correlation with other hematological and clinical prognostic factors (white blood cells [WBC] count, age, gender, central nervous system involvement, and response to treatment). PATIENTS, MATERIALS, AND METHODS: This cross-sectional study was conducted on 60 children; 20 patients with newly diagnosed ALL before starting induction therapy, 20 patients with ALL during remission (postinduction), and 20 healthy controls. Measurement of serum LEF1 level was done by enzyme-linked immunosorbent assay. RESULTS: Serum level of LEF1 was higher in newly diagnosed patients than in either patients at remission or controls with highly significant differences. There is a significant positive correlation with total WBC count and no significant correlation between LEF1 level and other hematological and clinical parameters or with immunophenotypic subtypes. There was no significant correlation between LEF1 serum level and response to remission induction. CONCLUSION: A high serum concentration of LEF1 is found in newly diagnosed patients with ALL and showed a significant positive correlation with total WBC count.
背景:急性淋巴母细胞白血病(Acute lymphoblastic leukemia, ALL)是一种异质性疾病,其特征是未成熟淋巴样细胞增生,在骨髓、外周血和髓外部位积聚,导致该疾病的临床表现。淋巴增强因子-1 (Lymphoid enher -binding factor-1, LEF1)是wingless型信号通路的靶基因和中枢介质,在正常造血中具有重要作用。据报道,在许多类型的血液和非血液恶性肿瘤中,LEF1的高表达是一种预后标志物。研究目的:评估小儿ALL患者血清LEF1水平及其与其他血液学和临床预后因素(白细胞计数、年龄、性别、中枢神经系统受累和对治疗的反应)的相关性。患者、材料和方法:这项横断面研究对60名儿童进行了研究;20例开始诱导治疗前新诊断的ALL患者,20例缓解期ALL患者(诱导后),20例健康对照。采用酶联免疫吸附法测定血清LEF1水平。结果:新诊断患者血清LEF1水平高于缓解期患者和对照组,且差异极显著。与白细胞总数有显著正相关,LEF1水平与其他血液学和临床参数及免疫表型亚型无显著相关。血清LEF1水平与缓解诱导反应无显著相关性。结论:初诊ALL患者血清中LEF1浓度较高,且与WBC总计数呈显著正相关。
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引用次数: 1
Febrile neutropenia risk factors in actively treated diffuse large B-cell lymphoma patients 积极治疗弥漫性大B细胞淋巴瘤患者的发热性中性粒细胞减少症危险因素
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_37_21
M. Bakırtaş, T. Yiğenoğlu, S. Başcı, B. Ulu, S. Yaman, M. Çakar, M. Dal, F. Altuntaş
BACKGROUND: Febrile neutropenia (FN) is a serious problem, especially in hematologic malignancies, and can cause high mortality rates and it occurs in 10%–20% of patients with lymphoma. The aim of this research is to assess the risk factors for FN, and the impact of FN on overall survival (OS) in patients with diffuse large B-cell lymphoma (DLBCL). MATERIALS AND METHODS: The study included 263 patients who were diagnosed with DLBCL and treated with mostly R-CHOP-based chemotherapy. Data including gender, age, Ann Arbor stage, International Prognostic Index (IPI) score, immunohistologic subtype, treatment regimens, response to treatment, and any FN episode were recorded. The factors predicting FN were analyzed. RESULTS: Significant predictors of FN were the number of chemotherapy lines received and IPI score. The median OS was significantly different between DLBCL patients who had at least one FN episode during the first-line chemotherapy and those who did not (P < 0,001). Significant predictors of OS in the multivariate analysis were the number of chemotherapy lines received, stage, Eastern Cooperative Oncology Group, and disease status. CONCLUSION: Our study reveals that OS is significantly shorter in patients who had an FN episode than those who did not. Therefore, it is crucial to demonstrate all factors related to FN to prevent FN episodes. In our study, the number of chemotherapy lines received and IPI score was found to be significant predictors of FN. Close follow-up should be done in these patients as the risk of FN is higher.
背景:发热性中性粒细胞减少症(FN)是一个严重的问题,尤其是在血液系统恶性肿瘤中,它会导致高死亡率,发生在10%-20%的淋巴瘤患者中。本研究的目的是评估FN的危险因素,以及FN对弥漫性大B细胞淋巴瘤(DLBCL)患者总生存期(OS)的影响。材料和方法:该研究包括263名被诊断为DLBCL并接受主要基于R-CHOP的化疗的患者。记录包括性别、年龄、Ann Arbor分期、国际预后指数(IPI)评分、免疫组织学亚型、治疗方案、治疗反应和任何FN发作在内的数据。分析预测FN的因素。结果:FN的重要预测因素是接受化疗的化疗线数和IPI评分。在一线化疗期间至少有一次FN发作的DLBCL患者和没有发生FN发作的患者之间,中位OS有显著差异(P<0.001)。在多变量分析中,OS的重要预测因素是接受化疗的行数、分期、东方肿瘤合作组和疾病状况。结论:我们的研究表明,有FN发作的患者的OS明显短于没有FN发作的病人。因此,证明所有与FN相关的因素对预防FN发作至关重要。在我们的研究中,接受化疗的线数和IPI评分被发现是FN的重要预测因素。由于FN的风险较高,应对这些患者进行密切随访。
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引用次数: 0
A comparative study of anemia in peripheral blood smear and automated cell counter generated red cell parameters 外周血涂片和自动细胞计数器生成红细胞参数检测贫血的比较研究
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_3_22
J. Phukan, Hena Kawsar, Jayashree Banerjee, Anuradha Sinha
BACKGROUND: Complete blood count and cell counter generated red blood cell (RBC) parameters help in morphological typing of anemia. Still the importance of microscopic examination of peripheral blood smears (PBSs) cannot be excluded to interpret the cause of anemia. AIMS AND OBJECTIVES: The present study was done to diagnose the type of anemia by examination of PBS and automated cell counter generated parameters and to compare the findings between these two methods. MATERIALS AND METHODS: During the 6-months study, 9981 anemic blood samples were evaluated. PBS findings and cell counter generated RBC parameters and histograms were evaluated and compared. RESULTS: Among 9941 samples, microcytic hypochromic anemia consists of the majority of cases (5048, 50.47%), followed by normocytic normochromic (2187, 21.97%), dimorphic (1297, 12.99%), and lastly, hemolytic anemia (722, 7.24%). Compared with RBC parameters and peripheral smear findings, dimorphic and hemolytic anemia showed significant difference (P < 0.0001). When compared with RBC histogram, 4747 (47.56%) cases showed left shift, 820 (8.21%) cases showed right shift, and 2278 (22.82%) cases showed normal bell-shaped curve suggesting microcytic, macrocytic, and normocytic normochromic anemia, respectively. 635 (6.90%) and 1447 (14.50%) number of cases showed bimodal and broad-base histogram suggesting dimorphic and hemolytic anemia, respectively. CONCLUSION: PBS examination along with RBC histogram study can be able to categorize the type of anemia in the majority of cases. Each method should be used as complementary to each other that increases diagnostic accuracy.
背景:全血细胞计数和细胞计数器生成的红细胞(RBC)参数有助于贫血的形态学分型。然而,不能排除外周血涂片(PBSs)的显微镜检查对解释贫血原因的重要性。目的和目的:本研究通过检查PBS和自动细胞计数器生成的参数来诊断贫血类型,并比较这两种方法的结果。材料和方法:在为期6个月的研究中,对9981份贫血血样进行了评估。评估并比较PBS结果和细胞计数器生成的RBC参数和直方图。结果:9941份样本中,微细胞性低铬性贫血占大多数(5048例,50.47%),其次是正常细胞性常铬性贫血(2187例,21.97%)、二型贫血(1297例,12.99%),最后是溶血性贫血(722例,7.24%)。与红细胞参数和外周血涂片检查结果相比,二型贫血和溶血性贫血有显著差异(P<0.0001),4747例(47.56%)显示左移,820例(8.21%)显示右移,2278例(22.82%)显示正常钟形曲线,分别提示小细胞、大细胞和正常细胞的常铬性贫血。635例(6.90%)和1447例(14.50%)分别显示双峰和宽基直方图,提示二型性和溶血性贫血。结论:PBS检查结合红细胞直方图研究可以对大多数病例的贫血类型进行分类。每种方法都应相互补充,以提高诊断准确性。
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引用次数: 0
Childhood acute lymphoblastic leukemia: Immunophenotypic profile and aberrant expression of CD13, CD33, CD117, CD11b, CD16, and CD64 儿童急性淋巴细胞白血病:CD13、CD33、CD117、CD11b、CD16和CD64的免疫表型特征和异常表达
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_36_21
Ihsan Al-Badran, Haithem A Al-Rubaie, Tamara Al-Assadi
BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) is the most prevalent malignant disease (25%–30%) and the most common type of leukemia (75%–80%) among children. It is not a single disease with significant phenotypic and genotypic variability that has diagnostic and prognostic implications. This study aims to provide the immunophenotypic profile of childhood ALL in Iraqi patients and to explore the frequency of aberrant myeloid antigen expression and their association with hematological parameters. PATIENTS, MATERIALS AND METHODS: The records of 67 pediatric patients diagnosed as ALL were reviewed for their flow cytometric immunophenotyping results at presentation. RESULTS: B-ALL constituted 76.1% of the cases and 23.9% were T-ALL. There was a highly significant statistical relation between higher age interval and T-ALL phenotypes (P = 0.001). Higher hemoglobin (Hb) level and white blood cell count were significantly related with T-ALL subtype (P = 0.039 and < 0.001, respectively). CD34, HLA-DR, CD10, and CD79a were significantly correlated with B-ALL compared to T-ALL (P = 0.007, <0.001, <0.001, and <0.001, respectively). With no significant differences, aberrant myeloid antigen expression was found in 51% of B-ALL and in 25% of T-ALL cases; however, CD34 expression was substantially related with aberrant myeloid antigen expression (P = 0.001). CONCLUSION: Aberrant myeloid antigens were expressed in 44.9% of ALL patients with insignificant differences between B- and T-ALL phenotypes. CD34 was significantly associated with B-lineage ALL and with aberrant myeloid antigen expression. T-ALL children are older and have significantly higher Hb concentration and white blood cell count. No correlation was found between aberrant myeloid expression and hematological parameters in B-ALL.
背景:儿童急性淋巴细胞白血病(ALL)是儿童中最常见的恶性疾病(25%-30%)和最常见的白血病类型(75%-80%)。它不是一种具有显著表型和基因型变异性的单一疾病,具有诊断和预后意义。本研究旨在提供伊拉克儿童ALL患者的免疫表型特征,探讨骨髓抗原异常表达的频率及其与血液学参数的关系。患者、材料和方法:对67例诊断为ALL的儿科患者的记录进行回顾,以了解他们在就诊时的流式细胞术免疫表型结果。结果:b型all占76.1%,t型all占23.9%。较高的年龄间隔与T-ALL表型之间存在高度显著的统计学关系(P = 0.001)。较高的血红蛋白(Hb)水平和白细胞计数与T-ALL亚型显著相关(P分别= 0.039和< 0.001)。与T-ALL相比,CD34、HLA-DR、CD10和CD79a与B-ALL的相关性显著(P分别为0.007、<0.001、<0.001和<0.001)。在51%的B-ALL和25%的T-ALL病例中发现异常的髓系抗原表达,差异无统计学意义;然而,CD34表达与异常髓系抗原表达有显著相关性(P = 0.001)。结论:44.9%的ALL患者有异常髓系抗原表达,B-型与t -型差异不显著。CD34与b系ALL和异常髓系抗原表达显著相关。T-ALL儿童年龄较大,Hb浓度和白细胞计数明显较高。B-ALL患者骨髓异常表达与血液学参数无相关性。
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引用次数: 1
Health-related quality of life in adults with sickle cell disease in Duhok-Iraq 杜霍克-伊拉克成人镰状细胞病患者的健康相关生活质量
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_15_22
Naz Tahir, N. Al-Allawi
BACKGROUND: Sickle cell disease (SCD) is the second-most common hemoglobin disorder in Duhok governorate. Health-related quality of life (HRQoL) instrument provides physicians with the patient's perspectives of their disease and thus more patient-oriented care. MATERIALS AND METHODS: In this case–control study, a total of 70 adults (≥18 years old) registered as SCD at the center for blood diseases in Duhok as well as 70 age- and sex-matched healthy controls were enrolled. Enrolled patients had their records and treatment reviewed, were clinically assessed, and appropriately investigated. All patients and controls had their HRQoL scored using the SF36 questionnaire, which consists of eight domains, namely physical function, role limitation physical, role limitation emotional, vitality, emotional well-being, social function, bodily pain, and general health perception. RESULTS: The mean standard deviation age of the SCD patients enrolled was 26.2 (8.9) years and included 32 males and 38 females. The patients had significantly lower HRQoL scores in all eight domains when compared to their matched controls. The most affected domain was general health. Within the patients' group, it was noted that HRQoL scores were negatively correlated with age in several domains, most significantly with general health (P = 0.011). On the other hand, there was no significant difference in HRQoL in relevance to gender, marital status, education, or employment. The most significant negative correlations of HRQoL scores were documented with the annual number of pain episodes and hospital admissions observed with all eight domains. CONCLUSIONS: The current study documented that in adults with SCD, HRQoL in all domains was significantly worse than in healthy controls and that it gets worse with age. The most significant contributors to the worse HRQoL are pain episodes and hospital admissions. The study underscores the importance of HRQoL assessments to enable attending physicians provide more patient-centered management.
背景:镰状细胞病(SCD)是杜霍克省第二常见的血红蛋白疾病。健康相关生活质量(HRQoL)仪器为医生提供患者对其疾病的看法,从而提供更多以患者为导向的护理。材料和方法:在这项病例对照研究中,共有70名成人(≥18岁)在Duhok血液疾病中心登记为SCD,以及70名年龄和性别匹配的健康对照。对入组患者的记录和治疗进行了回顾,进行了临床评估,并进行了适当的调查。所有患者和对照组均使用SF36问卷对HRQoL进行评分,该问卷由8个领域组成,即身体功能、角色限制身体、角色限制情感、活力、情感幸福感、社会功能、身体疼痛和一般健康感知。结果:纳入的SCD患者的平均标准差年龄为26.2(8.9)岁,包括32名男性和38名女性。与对照组相比,患者在所有8个领域的HRQoL评分均显著降低。受影响最大的域是“一般运行状况”。在患者组中,HRQoL评分在几个领域与年龄呈负相关,与一般健康状况呈显著负相关(P = 0.011)。另一方面,HRQoL与性别、婚姻状况、教育程度、就业相关无显著差异。HRQoL评分最显著的负相关与所有八个领域的年疼痛发作次数和住院次数有关。结论:目前的研究表明,SCD成人患者的HRQoL在所有领域都明显低于健康对照组,并且随着年龄的增长而恶化。导致HRQoL恶化的最重要因素是疼痛发作和住院。该研究强调了HRQoL评估的重要性,使主治医生能够提供更多以患者为中心的管理。
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引用次数: 0
A study on significance and correlation of red cell distribution width with severity of clinical illness in COVID-19 patients 新冠肺炎患者红细胞分布宽度与临床病情严重程度的相关性及意义
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_45_21
M. SakthiKannamma, B. Srinivasamurthy, S. Sinhasan, R. Bhat
BACKGROUND: Global pandemic COVID-19 is an acute respiratory illness with a high rate of hospitalization and death rate. Red cell distribution width coefficient of variation (RDW-CV), a routine component of complete blood count (CBC) automatically generated by most hematology analyzers is a useful predictor of clinical outcomes in critically ill patients and in those with infection and sepsis. RDW will provide information for early risk stratification of COVID-19 patients, thereby enabling timely intervention for reducing morbidity and mortality. In such a massive pandemic, early stratification of cases based on routinely available biomarkers can be of great help inefficient utilization of critical care and laboratory assets. MATERIALS AND METHODS: We retrospectively studied the significance and correlation of RDW (CV) (admission) with the severity of clinical illness in 800 confirmed cases of COVID-19 between August 2020 and October 2020 at our hospital. Demographic and clinical details were obtained from medical records; data pertaining to CBC were retrieved through electronic records of our fully automated hematology analyzer (NihonKoden 5 part auto analyzer Model-MEK– 7300K). Statistical workup was done and results were analyzed. RESULTS: Of 800 patients, 60% were male. RDW (CV) >14.5 (elevated) seen in 52% males and 47% females. Elevated RDW was noted in 43.6% (300/688) nonsevere illnesses (mild and moderate), 82% (92/112) in the severe illness group. The mean RDW (CV) for mild, moderate, and severe cases was found to be 14.21 ± 0.61, 15.32 ± 0.67, and 16.34 ± 1.64, respectively. The number of survivors was 704 (88%). The number of people who died was 96 (12%). Elevated RDW was seen in 74% (71/96) who died and 45% (321/704) of people who survived. To determine the efficacy of RDW (CV) in identifying the severity of disease, a ROC curve was used in which a cutoff value of 13.65 is obtained with a sensitivity of 97.3% and specificity of 85%. CONCLUSION: Higher RDW (CV) was found to have a significant association with clinical severity and mortality prediction. Hence, it can be considered as one of the important hematological parameters in the workup to efficiently stratify the patients at the earliest in COVID-19.
背景:全球大流行新冠肺炎是一种住院率和死亡率较高的急性呼吸道疾病。红细胞分布宽度变异系数(RDW-CV)是大多数血液学分析仪自动生成的全血细胞计数(CBC)的常规成分,是危重患者以及感染和败血症患者临床结果的有用预测指标。RDW将为新冠肺炎患者的早期风险分层提供信息,从而能够及时干预以降低发病率和死亡率。在这样一场大规模的流行病中,根据常规可用的生物标志物对病例进行早期分层,对重症监护和实验室资产的低效利用有很大帮助。材料与方法:回顾性研究了我院2020年8月至2020年10月收治的800例新冠肺炎确诊病例的RDW(CV)(入院)与临床疾病严重程度的相关性及其意义。从医疗记录中获得人口统计学和临床细节;通过我们的全自动血液分析仪(NihonKoden 5部件自动分析仪MEK-7300K型)的电子记录检索与CBC相关的数据。进行了统计工作,并对结果进行了分析。结果:800例患者中,60%为男性。52%的男性和47%的女性RDW(CV)>14.5(升高)。43.6%(300/688)的非严重疾病(轻度和中度)和82%(92/112)的严重疾病组RDW升高。轻度、中度和重度病例的平均RDW(CV)分别为14.21±0.61、15.32±0.67和16.34±1.64。幸存者人数为704人(88%)。死亡人数为96人(12%)。74%(71/96)的死亡者和45%(321/704)的幸存者的RDW升高。为了确定RDW(CV)在识别疾病严重程度方面的疗效,使用ROC曲线,其中获得13.65的截止值,灵敏度为97.3%,特异性为85%。结论:较高的RDW(CV)与临床严重程度和死亡率预测有显著相关性。因此,它可以被视为新冠肺炎早期有效分层患者的重要血液学参数之一。
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引用次数: 0
Basophil counting by hematology analyzers in cases of suspected chronic myeloid leukemia 怀疑慢性髓性白血病的血液分析仪的嗜碱性粒细胞计数
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_44_21
Johannes Hoffmann
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引用次数: 0
Comparison between H63D and G71D gene mutation effects on iron overload in Iraqi patients with β-thalassemia major: A case–control study H63D与G71D基因突变对伊拉克β-地中海贫血患者铁负荷影响的病例对照研究
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_10_22
Samar Maatooq, M. Alwash, A. Ahmed
BACKGROUND: Iron overload certainly will develop in β-thalassemia major. Iron homeostasis was mostly regulated by hepcidin that synthesized in the liver and encoded by the hepcidin antimicrobial peptide (HAMP) gene. HAMP and HFE genes, respectively, encode iron-regulating proteins (Hepcidin and HFE). The iron overload's possibility will increase if there is an interaction between β-thalassemia and HAMP-HFE gene mutations. AIM OF STUDY: In β-thalassemia major patients, we need to identify mutations in iron-regulating genes (HAMP and HFE genes), their impact on the iron overload, and their association with some clinicopathological parameters. PATIENTS AND METHODS: During a period of 5 months from (November 2020 to March 2021), a case–control study was conducted. It included 80 patients and controls aged ≥14 years and divided into two groups: thalassemic patient group included 40 patients who were diagnosed by complete blood count, blood film, and hemoglobin – electrophoresis as β-thalassemia major and control group included 40 unrelated, apparently healthy controls that were age and gender matched with thalassemic patient group. Complete blood count, liver and renal function tests, serum ferritin, and DNA extraction were performed. RESULTS: There was a statistically significant difference between study groups by H63D mutations. The proportion of CG genotype was significantly higher among thalassemic patient group than that in controls. There was no statistically significant difference (P = 0.082) between study groups by G71D mutations. Serum ferritin and Alanine transaminase (ALT) levels were significantly higher in patients with CG and GG genotypes compared to that in patients with CC genotype of H63D. CONCLUSION: H63D is associated with iron overload in β-thalassemia patients with unapparent effect on biochemical and hematological data except for ALT and serum ferritin. This could allow early diagnosis and proper treatment to overcome the complications of iron overload in those patients.
背景:铁超载肯定会在β-地中海贫血中发生。铁稳态主要由肝内合成的hepcidin抗菌肽(hepcidin antimicrobial peptide, HAMP)基因编码调控。HAMP和HFE基因分别编码铁调节蛋白(Hepcidin和HFE)。如果β-地中海贫血与HAMP-HFE基因突变之间存在相互作用,则铁超载的可能性会增加。研究目的:在β-地中海贫血重症患者中,我们需要确定铁调节基因(HAMP和HFE基因)的突变,它们对铁超载的影响,以及它们与一些临床病理参数的关联。患者和方法:在2020年11月至2021年3月的5个月期间,进行了一项病例对照研究。纳入80例年龄≥14岁的患者和对照组,分为两组:地中海贫血患者组包括40例经全血细胞计数、血膜、血红蛋白电泳诊断为β-地中海重度贫血的患者;对照组包括40例年龄、性别与地中海贫血患者组相匹配的无血缘关系、明显健康的对照组。进行全血细胞计数、肝肾功能检查、血清铁蛋白和DNA提取。结果:H63D突变组间差异有统计学意义。地中海贫血患者组CG基因型比例明显高于对照组。G71D突变组间差异无统计学意义(P = 0.082)。CG和GG基因型患者血清铁蛋白和丙氨酸转氨酶(ALT)水平明显高于H63D CC基因型患者。结论:H63D与β-地中海贫血患者铁超载有关,除ALT和血清铁蛋白外,对生化和血液学指标影响不明显。这可以使早期诊断和适当的治疗,以克服这些患者铁超载的并发症。
{"title":"Comparison between H63D and G71D gene mutation effects on iron overload in Iraqi patients with β-thalassemia major: A case–control study","authors":"Samar Maatooq, M. Alwash, A. Ahmed","doi":"10.4103/ijh.ijh_10_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_10_22","url":null,"abstract":"BACKGROUND: Iron overload certainly will develop in β-thalassemia major. Iron homeostasis was mostly regulated by hepcidin that synthesized in the liver and encoded by the hepcidin antimicrobial peptide (HAMP) gene. HAMP and HFE genes, respectively, encode iron-regulating proteins (Hepcidin and HFE). The iron overload's possibility will increase if there is an interaction between β-thalassemia and HAMP-HFE gene mutations. AIM OF STUDY: In β-thalassemia major patients, we need to identify mutations in iron-regulating genes (HAMP and HFE genes), their impact on the iron overload, and their association with some clinicopathological parameters. PATIENTS AND METHODS: During a period of 5 months from (November 2020 to March 2021), a case–control study was conducted. It included 80 patients and controls aged ≥14 years and divided into two groups: thalassemic patient group included 40 patients who were diagnosed by complete blood count, blood film, and hemoglobin – electrophoresis as β-thalassemia major and control group included 40 unrelated, apparently healthy controls that were age and gender matched with thalassemic patient group. Complete blood count, liver and renal function tests, serum ferritin, and DNA extraction were performed. RESULTS: There was a statistically significant difference between study groups by H63D mutations. The proportion of CG genotype was significantly higher among thalassemic patient group than that in controls. There was no statistically significant difference (P = 0.082) between study groups by G71D mutations. Serum ferritin and Alanine transaminase (ALT) levels were significantly higher in patients with CG and GG genotypes compared to that in patients with CC genotype of H63D. CONCLUSION: H63D is associated with iron overload in β-thalassemia patients with unapparent effect on biochemical and hematological data except for ALT and serum ferritin. This could allow early diagnosis and proper treatment to overcome the complications of iron overload in those patients.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45901402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Evaluation of the levels of the markers of ineffective erythropoiesis (transforming growth factor-beta, growth differentiation factor 15 and erythropoietin) in patient with ß-thalassemia syndrome and its correlation to clinical and hematological parameters ß-地中海贫血综合征患者无效红细胞生成标志物(转化生长因子- β、生长分化因子15和促红细胞生成素)水平的评价及其与临床和血液学参数的相关性
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_39_21
M. Yousif, H. Al-Mamoori
BACKGROUND: Anemia of β thalassemia results from a combination of ineffective erythropoiesis and hemolysis. This stimulates erythropoietin (EPO) production, which causes expansion of the bone marrow and may lead to serious deformities of the skull and long bones. Ineffective erythropoiesis also induces the release of growth differentiation factor 15 (GDF-15) and transforming growth factor-beta (TGF-β) which have been identified as regulators of hepcidin expression. OBJECTIVE: The objective is to evaluate the level of TGF-β, GDF-15, and EPO in patients with thalassemia syndrome. PATIENTS, MATERIALS AND METHODS: Patient samples were collected from Thalassemia Center of Ibn Al-Baladi Hospital. This study included 35 patients with thalassemia, 18 patients with beta-thalassemia major and 17 patients with beta-thalassemia intermedia. The age of studied group was 3–17 years. Twenty control healthy subjects were included for comparison who were age- and sex-matched with the patients group. Gel tube was used for collection of serum for enzyme-linked immunosorbent assay test for GDF-15, TGF-β, and EPO). RESULTS: There was a highly significant difference in GDF-15 and EPO levels among studied groups (P < 0.001). In addition, there was no significant difference in TGF-β level among studied groups (P > 0.05). TGF-β, GDF-15, and EPO were not significantly correlated to splenomegaly, hepatosplenomegaly, and frequency of blood transfusion duration in patients with beta-thalassemia major (P > 0.05), while TGF-β and EPO were significantly correlated to splenomegaly, hepatosplenomegaly in patients with beta-thalassemia intermedia but GDF-15 was not significantly correlated. In patients with beta-thalassemia major, EPO was negatively correlated to hemoglobin, packed cell volume, mean corpuscular volume, and red blood cells (RBC) count whereas GDF-15 significantly correlated to lymphocyte and neutrophil counts. TGF-β was significantly correlated to platelet count. In patients with beta-thalassemia intermedia, EPO and GDF-15 were not correlated to any hematological parameters whereas TGF-β was significantly correlated to RBC counts. CONCLUSION: Marker of erythropoiesis GDF-15, EPO was highly expressed in patient with beta-thalassemia major and beta-thalassemia intermedia as compared to the control group and this can be used as a future therapeutic goal for the suppression of ineffective erythropoiesis.
背景:β地中海贫血是红细胞生成无效和溶血综合作用的结果。这会刺激红细胞生成素(EPO)的产生,从而导致骨髓膨胀,并可能导致颅骨和长骨的严重畸形。无效的红细胞生成也会诱导生长分化因子15(GDF-15)和转化生长因子β(TGF-β)的释放,这两种因子已被确定为铁调素表达的调节因子。目的:评估地中海贫血综合征患者TGF-β、GDF-15和EPO的水平。患者、材料和方法:患者样本取自伊本·巴拉迪医院地中海贫血中心。这项研究包括35名地中海贫血患者、18名重型β地中海贫血患者和17名中间型β地中海贫血症患者。研究组的年龄为3-17岁。20名对照健康受试者被纳入比较,他们的年龄和性别与患者组相匹配。凝胶管用于收集血清进行GDF-15、TGF-β和EPO的酶联免疫吸附试验)。结果:研究组间GDF-15和EPO水平差异极显著(P<0.001)。此外,研究组间TGF-β水平无显著差异(P>0.05)。TGF-β、GDF-15、EPO与严重β地中海贫血患者的脾肿大、肝脾肿大和输血次数无显著相关性(P>05),TGF-β和EPO与中间型β地中海贫血患者的脾肿大、肝脾肿大显著相关,而GDF-15与脾肿大无关。在严重β地中海贫血患者中,EPO与血红蛋白、堆积细胞体积、平均红细胞体积和红细胞计数呈负相关,而GDF-15与淋巴细胞和中性粒细胞计数显著相关。TGF-β与血小板计数显著相关。在中间型β地中海贫血患者中,EPO和GDF-15与任何血液学参数均不相关,而TGF-β与红细胞计数显著相关。结论:与对照组相比,红细胞生成标志物GDF-15、EPO在重型β地中海贫血和中间型β地中海贫血患者中高表达,这可作为抑制无效红细胞生成的未来治疗目标。
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引用次数: 0
Seroprevalence of transfusion-transmissible infections among blood donors and their notification: A study from North India 献血者中输血传播感染的血清流行率及其通报:来自印度北部的一项研究
IF 0.1 Pub Date : 2022-01-01 DOI: 10.4103/ijh.ijh_14_22
S. Mandal, Ranjan Kumar, Deepika Parwan, Nem Singh, Richa Sharma, Bhaskar C. Das
BACKGROUND: Blood transfusion carries the risk of transfusion-transmissible infections (TTIs) if not properly screened. As per protocol blood donors who are found reactive for TTIs are requested to come for counseling and directed for further management. Many of them are either not interested or do not follow-up their visit to blood center. This study is undertaken to determine the rate of seroprevalence of TTIs and the attitude of reactive blood donors in response to post donation notification and counseling. MATERIALS AND METHODOLOGY: This observational study considers the blood donations from January 2019 to April 2021. Blood donors with reactive test results identified by different TTIs markers were notified, and their response rates were evaluated. RESULTS: During this study, 8904 donations were recorded out of which 171 donors were found to be reactive (1.92%), only 142 donors were contacted (89.30%), and only 74 reactive donors could be counseled (52.11%). CONCLUSION: This study shows low prevalence of TTI reactivity among blood donors and recommends strengthening of donor notification and counseling practices in blood centers and raises the question of need for central notification system for the traceability of reactive blood donors to prevent the spread of TTIs in the community.
背景:如果不进行适当的筛查,输血具有输血传播感染(tti)的风险。根据协议,被发现对tti有反应的献血者被要求前来咨询并指导进一步的管理。他们中的许多人要么不感兴趣,要么不去血液中心随访。本研究旨在确定tti的血清阳性率和反应性献血者对献血后通知和咨询的态度。材料与方法:本观察性研究考虑2019年1月至2021年4月的献血者。通过不同的tti标记物鉴定出反应性检测结果的献血者被通知,并评估他们的反应率。结果:本研究共记录8904例献血者,其中发现反应性献血者171例(1.92%),联系献血者142例(89.30%),咨询反应性献血者74例(52.11%)。结论:本研究显示献血者TTI反应性患病率较低,建议加强血液中心的献血者通报和咨询实践,并提出了需要建立中央通报系统以追踪反应性献血者以防止TTI在社区传播的问题。
{"title":"Seroprevalence of transfusion-transmissible infections among blood donors and their notification: A study from North India","authors":"S. Mandal, Ranjan Kumar, Deepika Parwan, Nem Singh, Richa Sharma, Bhaskar C. Das","doi":"10.4103/ijh.ijh_14_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_14_22","url":null,"abstract":"BACKGROUND: Blood transfusion carries the risk of transfusion-transmissible infections (TTIs) if not properly screened. As per protocol blood donors who are found reactive for TTIs are requested to come for counseling and directed for further management. Many of them are either not interested or do not follow-up their visit to blood center. This study is undertaken to determine the rate of seroprevalence of TTIs and the attitude of reactive blood donors in response to post donation notification and counseling. MATERIALS AND METHODOLOGY: This observational study considers the blood donations from January 2019 to April 2021. Blood donors with reactive test results identified by different TTIs markers were notified, and their response rates were evaluated. RESULTS: During this study, 8904 donations were recorded out of which 171 donors were found to be reactive (1.92%), only 142 donors were contacted (89.30%), and only 74 reactive donors could be counseled (52.11%). CONCLUSION: This study shows low prevalence of TTI reactivity among blood donors and recommends strengthening of donor notification and counseling practices in blood centers and raises the question of need for central notification system for the traceability of reactive blood donors to prevent the spread of TTIs in the community.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46870155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
期刊
Iraqi Journal of Hematology
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