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Impact of aberrant antigens expression on remission rate after first induction course of chemotherapy in de novo adult acute myeloid leukemia 异常抗原表达对新生成人急性髓性白血病首次诱导化疗后缓解率的影响
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_17_21
G. Hussein, Ali H. Jawad
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引用次数: 1
Transfusion support in a severe autoimmune hemolytic anemia patient associated with systemic lupus erythematosus and antiphospholipid syndrome 输血支持与系统性红斑狼疮和抗磷脂综合征相关的严重自身免疫性溶血性贫血患者
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_8_21
Debasish Mishra, G. Kanungo, M. Agrawal, Aditi Khanna
A positive direct antiglobulin test is a criterion for the diagnosis of systemic lupus erythematosus (SLE). In general, severe hemolysis is absent in SLE. Sometimes, these patients may show hemolysis when presenting with antiphospholipid syndrome (APS). It is essential to exclude an underlying alloantibody along with autoantibody. We had reported a case of a 24-year-old female SLE along with an APS patient requiring transfusion support with underlying allo-anti-S antibody. We provided two units of S antigen-negative best-matched units to the patient who tolerated it well and showed improvement.
直接抗球蛋白试验阳性是诊断系统性红斑狼疮(SLE)的标准。一般来说,SLE患者没有严重的溶血。有时,这些患者在出现抗磷脂综合征(APS)时可能会出现溶血。排除同种异体抗体和自身抗体是必要的。我们报告了一例24岁女性SLE伴APS患者,需要输血支持,伴有同种异体抗s抗体。我们提供了两个单位的S抗原阴性最佳匹配单位的患者耐受性良好,并显示改善。
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引用次数: 0
Serum lactate dehydrogenase level in childhood acute lymphoblastic leukemia 儿童急性淋巴细胞白血病患者血清乳酸脱氢酶水平
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_4_21
S. Zahra, Entisar Al-Shammary, Ishraq Hameed
BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. It accounts for one-fourth of all childhood cancers and 72% of all cases of childhood leukemia. OBJECTIVE: The objective was to evaluate the significant increase in serum lactate dehydrogenase (LDH) enzyme levels in patients with ALL with respect to patients' characters, clinical presentation, and patients' induction outcome. PATIENTS AND METHODS: A prospective study had been conducted during the period from November 1, 2017 to October 31, 2018, included 86 patients newly diagnosed ALL patients under the age of 15th years, admitted to the pediatric hemato-oncology unit in the Child's Central Teaching Hospital the data were collect from the patients, included, age, sex, clinical presentation, investigation and induction outcome of ALL patients to undergo analysis of study. RESULTS: Of a total (86) ALL patients started induction therapy, only (75/86) of them completed induction phase of therapy and those were enrolled in analysis of this study, while (11/86) did not complete induction therapy and excluded from the study (because 10 died, and one patient loss follow-up during induction). The mean age was 4.7 years. The male-to-female ratio was 1.26:1. LDH level ranged from 100 to 1995 U/L. There was a significant association between LDH level at day 0 and each of age and ALL risk group and no association with gender, hepatomegaly, splenomegaly, lymphadenopathy, central nervous system status, and induction outcome (remission/no remission). The mean of LDH levels at diagnosis was highly elevated in patients with ALL (726 ± 422 U/L); the response to induction treatment was observed by the significant decrease in mean LDH level (324 ± 201 U/L) at day 28th of treatment P value (0.0001). CONCLUSIONS: The serum LDH level was highly elevated at diagnosis in the majority of ALL patients and decreased significantly in response to chemotherapy. The estimation of serum LDH level is easy, and available, so it may be a helpful tool in evaluating the clinical aspect of the disease, the response to induction chemotherapy.
背景:急性淋巴细胞白血病(ALL)是儿童最常见的恶性肿瘤。它占所有儿童癌症的四分之一和所有儿童白血病病例的72%。目的:目的是评估ALL患者血清乳酸脱氢酶(LDH)水平的显著升高与患者特征、临床表现和患者诱导结局的关系。患者与方法:前瞻性研究于2017年11月1日至2018年10月31日在儿童中心教学医院儿科血液肿瘤科收治的86例15岁以下新诊断ALL患者,收集患者资料,包括ALL患者的年龄、性别、临床表现、调查及诱导结果,对研究进行分析。结果:全部(86)例患者中,只有(75/86)例患者完成了诱导期治疗并入选本研究分析,而(11/86)例患者未完成诱导期治疗,因10例患者死亡,1例患者在诱导期失去随访而被排除在本研究之外。平均年龄为4.7岁。男女比例为1.26:1。LDH水平为100 ~ 1995 U/L。第0天LDH水平与各年龄和ALL危险组之间存在显著相关性,与性别、肝肿大、脾肿大、淋巴结病变、中枢神经系统状态和诱导结局(缓解/无缓解)无关。ALL患者诊断时LDH水平均值高(726±422 U/L);诱导处理后第28天LDH水平显著降低(324±201 U/L), P值为0.0001。结论:大多数ALL患者诊断时血清LDH水平高,化疗后显著降低。血清乳酸脱氢酶水平的测定是一种简单易行的方法,因此它可能是评估该疾病的临床方面以及对诱导化疗反应的有用工具。
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引用次数: 0
Impact of clinical staging and demographic data (age and sex) on response to treatment and survival of chronic lymphocytic leukemia patients in Kurdistan Region of Iraq 伊拉克库尔德斯坦地区慢性淋巴细胞白血病患者的临床分期和人口统计学数据(年龄和性别)对治疗反应和生存的影响
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_11_21
BryarSabah Rashid, S. Jalal, Ahmed K. Yassin, Kawa Hassan, Z. Mohamed, Zhala Ahmed, Tavan I. Mahmood, Rawand P. Shamoon, S. Mustafa, M. Karam, D. Abdullah, ShlanS Mohammad, L. Abdulrahman, Rozh-hatA Yousif, Basil K. Abdulla, N. Mohammed, H. Getta, R. Polus, G. Numan
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引用次数: 2
Impact of malaria co-infection on leukocyte indices of tuberculosis-infected participants at pretreatment, intensive, and continuation phase anti-tuberculosis therapy 疟疾合并感染对结核感染者抗结核治疗前、强化和继续期白细胞指数的影响
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_58_20
C. Okeke, M. Ifeanyichukwu, C. Okeke, S. Ibekailo, Sunday Ogamde
BACKGROUND: Hematological changes involving all blood cells are some of the most common complications in both tuberculosis (TB) and malaria infection. The changes induced by malaria infection are diverse, and the first line anti-TB treatment regimen which involves two phases may alter these changes in TB participants co-infected with malaria (TB/MP). OBJECTIVE: In this study, we aimed to ascertain the impact of malaria co-infection on leukocyte indices of TB-infected participants at pre-treatment, intensive and continuation phase therapy. MATERIALS AND METHODS: In this cross-sectional study, 180 participants were recruited comprising; 60 (35 TB and 25 TB malaria) participants before treatment, sixty (36 TB and 24 TB-Malaria) participants after intensive phase treatment and sixty (27 TB and 33 TB-Malaria) participants after continuation phase therapy. Whole blood was used for the measurement of total (total white blood cell [TWBC]) and differential white cell count, Platelet count, and packed cell volume (PCV). RESULTS: Before initiation of treatment, TWBC, neutrophil, lymphocyte, platelet count, and neutrophil-lymphocyte ratio were significantly reduced (P = 0.041, 0.022, 0.046, and 0.026, respectively), whereas eosinophil count was significantly increased in TB/Malaria participants compared to TB participants (P = 0.043). There was no significant change in these parameters after intensive phase treatment (P > 0.05). However, after continuation phase treatment, PCV was significantly reduced, while eosinophil was significantly increased in TB/Malaria participants compared with TB participants (P = 0.046 and 0.045, respectively). CONCLUSION: Malaria co-infection induces the significant reduction in leukocyte indices of TB patients at pretreatment but not at the intensive and continuation phase anti-TB therapy except eosinophils count which was increased before treatment and continuation phase treatment.
背景:涉及所有血细胞的血液学改变是结核病和疟疾感染中最常见的并发症。疟疾感染引起的变化是多种多样的,包括两个阶段的一线抗结核治疗方案可能会改变合并感染疟疾的结核病参与者(TB/MP)的这些变化。目的:在本研究中,我们旨在确定疟疾合并感染对结核病感染者在治疗前、强化治疗和继续治疗阶段白细胞指数的影响。材料与方法:在这项横断面研究中,招募了180名参与者,包括;治疗前60名(35名结核和25名结核疟疾)参与者,强化阶段治疗后60名(36名结核和24名结核-疟疾)参与者,继续阶段治疗后60名(27名结核和33名结核-疟疾)参与者。全血用于测定总白细胞(total white blood cell, TWBC)和差异白细胞计数、血小板计数和堆积细胞体积(packed cell volume, PCV)。结果:治疗开始前,结核/疟疾患者的TWBC、中性粒细胞、淋巴细胞、血小板计数和中性粒细胞/淋巴细胞比值显著降低(P分别为0.041、0.022、0.046和0.026),而嗜酸性粒细胞计数显著高于结核患者(P = 0.043)。强化分期治疗后,这些参数均无显著变化(P < 0.05)。然而,在持续期治疗后,结核/疟疾患者的PCV显著减少,而嗜酸性粒细胞显著增加(P分别= 0.046和0.045)。结论:疟疾合并感染可导致结核患者白细胞指数在治疗前显著降低,但在强化和继续治疗阶段,除嗜酸性粒细胞计数在治疗前和继续治疗阶段升高外,其余白细胞指数在治疗前和继续治疗阶段均无显著降低。
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引用次数: 0
Serum ferritin and hematological indices in thalassemia minor and nontransfusion dependent hemoghlobinopathy 轻度地中海贫血和非输血依赖性血红蛋白病的血清铁蛋白和血液学指标
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_18_20
M. Dehghani, P. Karimzadeh, Nazanin Azadeh, A. Rezvani, Ali Kashkooe
BACKGROUND: Iron deficiency is not common in thalassemia minor and nontransfusion dependent hemoglobinophaties. The majority of these patients have normal-to-high serum ferritin. OBJECTIVES: The aims of the study were to evaluate serum ferritin levels in alpha and beta thalassemia minor and intermedia and in, hemoglobin H disease, and sickle cell anemia, and to investigate the effect of iron consumption on increasing serum ferritin levels and the role of Mentzer and Srivastava indices in patients with thalassemia minor and low serum ferritin levels. MATERIALS AND METHODS: In this study, 204 patients with alpha-thalassemia minor, beta-thalassemia minor, nontransfusion-dependent thalassemia intermediate, and sickle cell disease were studied. Serum ferritin levels, Mentzer, Srivastava, and Bordbar's formula were measured using erythrocyte indices. RESULTS: Irrespective of iron deficiency status, which was 5.9% and was more common in women, total iron intake was 39%. Iron deficiency status was 3.3% in alpha thalassemia, 9.8% in beta-thalassemia, and 4.5% in sickle cell disease. High and very high serum ferritin levels are more common in beta intermediate thalassemia and sickle cell anemia. Mentzer and Srivastava indices were not significant for differentiating thalassemia minor and iron deficiency, but the Bordbar's formula in thalassemia minor with iron deficiency was statistically significant (119.75). CONCLUSION: Patients with minor thalassemia and nontransfusion dependent hemoglobinopathy had a lower prevalence of iron deficiency according due to due to serum ferritin levels compared to the general population. High and very high ferritin is more common in intermediate thalassemia, hemoglobin H, and sickle cell patients.
背景:缺铁在轻度地中海贫血和非转运依赖性血红蛋白血症中并不常见。这些患者中的大多数具有正常至高血清铁蛋白。目的:本研究的目的是评估轻度和中度地中海贫血、血红蛋白H病和镰状细胞性贫血患者的血清铁蛋白水平,并研究铁消耗对轻度地中海贫血和低血清铁蛋白患者血清铁蛋白升高的影响以及Mentzer和Srivastava指数的作用。材料和方法:在本研究中,研究了204名患有轻度α地中海贫血、轻度β地中海贫血、非转运依赖性地中海贫血中间型和镰状细胞病的患者。使用红细胞指数测量血清铁蛋白水平、Mentzer、Srivastava和Bordbar公式。结果:不考虑缺铁状态(5.9%,在女性中更常见),总铁摄入量为39%。α地中海贫血的缺铁率为3.3%,β地中海贫血为9.8%,镰状细胞病为4.5%。高和极高的血清铁蛋白水平在β-中级地中海贫血和镰状细胞贫血中更常见。Mentzer和Srivastava指数在区分轻度地中海贫血和缺铁方面不显著,但Bordbar公式在伴有缺铁的轻度地中海贫血中具有统计学意义(119.75)。结论:与普通人群相比,由于血清铁蛋白水平,轻度地中海贫血和非转运依赖性血红蛋白病患者的缺铁患病率较低。高铁蛋白和极高铁蛋白在中度地中海贫血、血红蛋白H和镰状细胞病患者中更常见。
{"title":"Serum ferritin and hematological indices in thalassemia minor and nontransfusion dependent hemoghlobinopathy","authors":"M. Dehghani, P. Karimzadeh, Nazanin Azadeh, A. Rezvani, Ali Kashkooe","doi":"10.4103/ijh.ijh_18_20","DOIUrl":"https://doi.org/10.4103/ijh.ijh_18_20","url":null,"abstract":"BACKGROUND: Iron deficiency is not common in thalassemia minor and nontransfusion dependent hemoglobinophaties. The majority of these patients have normal-to-high serum ferritin. OBJECTIVES: The aims of the study were to evaluate serum ferritin levels in alpha and beta thalassemia minor and intermedia and in, hemoglobin H disease, and sickle cell anemia, and to investigate the effect of iron consumption on increasing serum ferritin levels and the role of Mentzer and Srivastava indices in patients with thalassemia minor and low serum ferritin levels. MATERIALS AND METHODS: In this study, 204 patients with alpha-thalassemia minor, beta-thalassemia minor, nontransfusion-dependent thalassemia intermediate, and sickle cell disease were studied. Serum ferritin levels, Mentzer, Srivastava, and Bordbar's formula were measured using erythrocyte indices. RESULTS: Irrespective of iron deficiency status, which was 5.9% and was more common in women, total iron intake was 39%. Iron deficiency status was 3.3% in alpha thalassemia, 9.8% in beta-thalassemia, and 4.5% in sickle cell disease. High and very high serum ferritin levels are more common in beta intermediate thalassemia and sickle cell anemia. Mentzer and Srivastava indices were not significant for differentiating thalassemia minor and iron deficiency, but the Bordbar's formula in thalassemia minor with iron deficiency was statistically significant (119.75). CONCLUSION: Patients with minor thalassemia and nontransfusion dependent hemoglobinopathy had a lower prevalence of iron deficiency according due to due to serum ferritin levels compared to the general population. High and very high ferritin is more common in intermediate thalassemia, hemoglobin H, and sickle cell patients.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":"10 1","pages":"17 - 22"},"PeriodicalIF":0.1,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45126845","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
Factor VII deficiency-related recurrent hemarthrosis in a female child – When to suspect? 因子7缺乏相关的女性儿童复发性关节病-何时怀疑?
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_6_21
S. Didel, A. Purohit, D. Krishna, Varuna Vyas, Kuldeep Singh
Recurrent hemarthrosis is a common entity in children. Although recurrent hemarthrosis most often associated with hemophilia (VIII or IX deficiency), but rarely it can be associated with factor VII deficiency (FVIID). It is a strong mimicker of hemophilic hemarthrosis. Once hemophilia is excluded as a cause of recurrent hemarthrosis, congenital FVIID needs to be considered for long-term planning of treatment and avoiding unnecessary transfusion of factor concentrates. Clinical presentation of FVIID has a varied spectrum and does not correlate with factor levels. Here, we present a case of recurrent hemarthrosis secondary to FVIID.
复发性关节血肿是儿童常见的疾病。虽然复发性血肿最常与血友病(VIII或IX缺乏)相关,但很少与因子VII缺乏(FVIID)相关。它是一种强烈的血友病模仿者。一旦排除血友病作为复发性血肿的原因,需要考虑先天性FVIID的长期治疗计划,避免不必要的输血浓缩因子。FVIID的临床表现多种多样,与因子水平无关。在此,我们报告一例继发于FVIID的复发性关节血肿。
{"title":"Factor VII deficiency-related recurrent hemarthrosis in a female child – When to suspect?","authors":"S. Didel, A. Purohit, D. Krishna, Varuna Vyas, Kuldeep Singh","doi":"10.4103/ijh.ijh_6_21","DOIUrl":"https://doi.org/10.4103/ijh.ijh_6_21","url":null,"abstract":"Recurrent hemarthrosis is a common entity in children. Although recurrent hemarthrosis most often associated with hemophilia (VIII or IX deficiency), but rarely it can be associated with factor VII deficiency (FVIID). It is a strong mimicker of hemophilic hemarthrosis. Once hemophilia is excluded as a cause of recurrent hemarthrosis, congenital FVIID needs to be considered for long-term planning of treatment and avoiding unnecessary transfusion of factor concentrates. Clinical presentation of FVIID has a varied spectrum and does not correlate with factor levels. Here, we present a case of recurrent hemarthrosis secondary to FVIID.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":"10 1","pages":"82 - 83"},"PeriodicalIF":0.1,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42145345","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
Understanding mixed phenotypic acute leukemia: A conundrum of six cases with review of literature 了解混合表型急性白血病:六个病例的难题与文献回顾
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_19_21
Sarika Singh, Nimisha Dhankar, P. Sahi, Richa Gupta, P. Sinha, Vishal Singh
{"title":"Understanding mixed phenotypic acute leukemia: A conundrum of six cases with review of literature","authors":"Sarika Singh, Nimisha Dhankar, P. Sahi, Richa Gupta, P. Sinha, Vishal Singh","doi":"10.4103/ijh.ijh_19_21","DOIUrl":"https://doi.org/10.4103/ijh.ijh_19_21","url":null,"abstract":"","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":"1 1","pages":""},"PeriodicalIF":0.1,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70749045","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
Congenital factor XIII deficiency in Iraq: An 8-year single-center study 伊拉克先天性XIII因子缺乏症:一项为期8年的单中心研究
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_1_21
A. Salih, K. Masood, E. Ibraheem
BACKGROUND: Congenital FXIII deficiency is a rare genetic bleeding disorder that is inherited in an autosomal recessive pattern with a frequency of 1/2 million individuals in the human population. Deficiency of FXIII is associated with significant bleeding disorders. AIMS: This study aimed to evaluate the demographic parameters, clinical presentations, and outcome of patients who were diagnosed with congenital factor XIII deficiency. SUBJECTS AND METHODS: A retrospective descriptive study of patients who were diagnosed with congenital FXIII deficiency over a period from August 2008 to August 2016 was conducted. The study included patients who were diagnosed by having bleeding tendency and normal standard coagulation tests (normal platelet count, normal prothrombin time; normal partial thromboplastin time, and normal bleeding time) and the diagnosis was confirmed by clot solubility test in 5M urea. The diagnosis was made in the Hemophilia Ward, Children Welfare Teaching Hospital, Medical City, Baghdad. RESULTS: There were 111 cases of other coagulation factors' deficiency (rare bleeding disorders) registered in the center, congenital FXIII deficiency represented 24 (22%) of them. Males represented 14 (58.3%) and females 10 (41.7%) of patients. Most of the patients (41.7%) had their symptoms during the 1st year of life. Positive consanguinity was found in 100% of patients and 14 (58.3%) patients had a family history of FXIII deficiency. Umbilical cord bleeding and gum bleeding were present in 37.5% and 20.8%, respectively, and they were the most common first presenting symptoms of FXIII-deficient patients, while muscle hematoma (28.5%) and joint bleeding (24.7%) were the most common presenting symptoms in follow-up visits. The majority of the patients (79.1%) did not develop complications, the complications were developed in 3 (12.4%) patients, which were intracranial bleeding in 2 (8.3%) patients and liver hematoma in 1 (4.1%) patient. One patient (4.1%) developed recurrent abortion and one patient (4.1%) developed hepatitis C. No death was reported during the study period. CONCLUSIONS: FXIII deficiency founded to be a more common rare bleeding disorder among Iraqi patients, with a high rate of consanguineous marriage. Umbilical cord bleeding and gum bleeding were the most common presenting symptoms for FXIII deficiency. There was a considerable delay in the diagnosis of FXIII deficiency in the majority of patients.
背景:先天性FXIII缺乏症是一种罕见的遗传性出血性疾病,以常染色体隐性遗传模式遗传,在人类中的发病率为1/2百万。FXIII缺乏与严重的出血性疾病有关。目的:本研究旨在评估被诊断为先天性因子XIII缺乏症患者的人口统计学参数、临床表现和预后。受试者和方法:对2008年8月至2016年8月期间被诊断为先天性FXIII缺乏症的患者进行回顾性描述性研究。该研究包括通过出血倾向和正常标准凝血测试(正常血小板计数、正常凝血酶原时间、正常部分凝血活酶时间和正常出血时间)诊断的患者,并通过在5M尿素中的凝块溶解度测试确认诊断。诊断是在巴格达医疗城儿童福利教学医院血友病病房做出的。结果:该中心登记的其他凝血因子缺乏症(罕见出血性疾病)有111例,其中先天性FXIII缺乏症占24例(22%)。男性占14例(58.3%),女性占10例(41.7%)。大多数患者(41.7%)在生命的第一年出现症状。在100%的患者中发现阳性血亲,14名(58.3%)患者有FXIII缺乏症家族史。脐带出血和牙龈出血分别占37.5%和20.8%,它们是FXIII缺陷患者最常见的首次症状,而肌肉血肿(28.5%)和关节出血(24.7%)是随访中最常见的症状。大多数患者(79.1%)没有出现并发症,3例(12.4%)出现并发症,其中2例(8.3%)出现颅内出血,1例(4.1%)出现肝血肿。一名患者(4.1%)发生复发性流产,一名患者发生丙型肝炎。研究期间未报告死亡。结论:FXIII缺乏症在伊拉克患者中是一种更常见的罕见出血性疾病,近亲结婚率高。脐带出血和牙龈出血是FXIII缺乏症最常见的症状。在大多数患者中,FXIII缺乏症的诊断有相当大的延迟。
{"title":"Congenital factor XIII deficiency in Iraq: An 8-year single-center study","authors":"A. Salih, K. Masood, E. Ibraheem","doi":"10.4103/ijh.ijh_1_21","DOIUrl":"https://doi.org/10.4103/ijh.ijh_1_21","url":null,"abstract":"BACKGROUND: Congenital FXIII deficiency is a rare genetic bleeding disorder that is inherited in an autosomal recessive pattern with a frequency of 1/2 million individuals in the human population. Deficiency of FXIII is associated with significant bleeding disorders. AIMS: This study aimed to evaluate the demographic parameters, clinical presentations, and outcome of patients who were diagnosed with congenital factor XIII deficiency. SUBJECTS AND METHODS: A retrospective descriptive study of patients who were diagnosed with congenital FXIII deficiency over a period from August 2008 to August 2016 was conducted. The study included patients who were diagnosed by having bleeding tendency and normal standard coagulation tests (normal platelet count, normal prothrombin time; normal partial thromboplastin time, and normal bleeding time) and the diagnosis was confirmed by clot solubility test in 5M urea. The diagnosis was made in the Hemophilia Ward, Children Welfare Teaching Hospital, Medical City, Baghdad. RESULTS: There were 111 cases of other coagulation factors' deficiency (rare bleeding disorders) registered in the center, congenital FXIII deficiency represented 24 (22%) of them. Males represented 14 (58.3%) and females 10 (41.7%) of patients. Most of the patients (41.7%) had their symptoms during the 1st year of life. Positive consanguinity was found in 100% of patients and 14 (58.3%) patients had a family history of FXIII deficiency. Umbilical cord bleeding and gum bleeding were present in 37.5% and 20.8%, respectively, and they were the most common first presenting symptoms of FXIII-deficient patients, while muscle hematoma (28.5%) and joint bleeding (24.7%) were the most common presenting symptoms in follow-up visits. The majority of the patients (79.1%) did not develop complications, the complications were developed in 3 (12.4%) patients, which were intracranial bleeding in 2 (8.3%) patients and liver hematoma in 1 (4.1%) patient. One patient (4.1%) developed recurrent abortion and one patient (4.1%) developed hepatitis C. No death was reported during the study period. CONCLUSIONS: FXIII deficiency founded to be a more common rare bleeding disorder among Iraqi patients, with a high rate of consanguineous marriage. Umbilical cord bleeding and gum bleeding were the most common presenting symptoms for FXIII deficiency. There was a considerable delay in the diagnosis of FXIII deficiency in the majority of patients.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":"10 1","pages":"65 - 68"},"PeriodicalIF":0.1,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43504097","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
Incidental detection of malaria parasite in automated hematology 6-Differential analyzer 自动血液学6-微分分析仪中疟原虫的偶然检测
IF 0.1 Q4 HEMATOLOGY Pub Date : 2021-01-01 DOI: 10.4103/ijh.ijh_57_20
S. Bhakta, T. Santosh, Arundhathi Shankaralingappa, L. Jamir
India has been facing the problem of malaria for centuries. Peripheral smear microscopy remains the gold standard for diagnosis; however, it is a tedious process and requires qualified staff. Flow cytometric-based hematology analyzers' scattergrams can be of vital use in identifying the abnormal scattergrams for malaria. We report a patient with fever and chills who presented to the outpatient department and was further evaluated for pyrexia of unknown origin. 6-Diff complete blood count analyzer incidentally showed abnormal scattergram which was evaluated by a pathologist and confirmed the presence of Plasmodium vivax. The accuracy in detection of malaria diagnosis can vary based on species, parasite load, immunity, and clinical context. Pathologist and technical staff should analyze any abnormal scattergram and hematological data along with peripheral smear to identify the parasites.
几个世纪以来,印度一直面临疟疾问题。外周涂片显微镜检查仍然是诊断的金标准;然而,这是一个乏味的过程,需要合格的工作人员。基于流式细胞仪的血液分析仪散点图在识别疟疾异常散点图方面具有重要用途。我们报告了一名发热和发冷的患者,他到门诊就诊,并进一步评估了不明原因的发热。6-Diff全血细胞计数分析仪偶然显示异常散点图,该散点图由病理学家评估并确认间日疟原虫的存在。疟疾诊断检测的准确性可能因物种、寄生虫载量、免疫力和临床情况而异。病理学家和技术人员应分析任何异常散点图和血液学数据以及外周涂片,以识别寄生虫。
{"title":"Incidental detection of malaria parasite in automated hematology 6-Differential analyzer","authors":"S. Bhakta, T. Santosh, Arundhathi Shankaralingappa, L. Jamir","doi":"10.4103/ijh.ijh_57_20","DOIUrl":"https://doi.org/10.4103/ijh.ijh_57_20","url":null,"abstract":"India has been facing the problem of malaria for centuries. Peripheral smear microscopy remains the gold standard for diagnosis; however, it is a tedious process and requires qualified staff. Flow cytometric-based hematology analyzers' scattergrams can be of vital use in identifying the abnormal scattergrams for malaria. We report a patient with fever and chills who presented to the outpatient department and was further evaluated for pyrexia of unknown origin. 6-Diff complete blood count analyzer incidentally showed abnormal scattergram which was evaluated by a pathologist and confirmed the presence of Plasmodium vivax. The accuracy in detection of malaria diagnosis can vary based on species, parasite load, immunity, and clinical context. Pathologist and technical staff should analyze any abnormal scattergram and hematological data along with peripheral smear to identify the parasites.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":"10 1","pages":"87 - 89"},"PeriodicalIF":0.1,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41639565","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
期刊
Iraqi Journal of Hematology
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