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Assessment of Erythrocyte Osmotic Fragility and Its Determinants, and Comparison of Hematological Indices Among Type 2 Diabetes Mellitus Patients on Follow-Up at Jimma Medical Center, Southwest Ethiopia. 埃塞俄比亚西南部吉马医疗中心随访的 2 型糖尿病患者红细胞渗透脆性及其决定因素的评估以及血液指标的比较。
IF 2 Q3 Medicine Pub Date : 2024-01-22 eCollection Date: 2024-01-01 DOI: 10.2147/JBM.S445620
Zenebe Tujara, Wondu Reta, Eyasu Tadesse, Ifa Dereje, Mekonnen Tesfa

Background: Diabetes Mellitus (DM) is one of the most prevalent non-communicable diseases worldwide. Approximately 9.3% of the general population was estimated to have DM globally in 2019. Erythrocyte osmotic fragility (EOF) in hyperglycemic patients is expected to increase and determine the rate of erythrocyte hemolysis.

Purpose: This study aimed to assess erythrocyte osmotic fragility (EOF) and its determinants and to compare hematological indices among T2DM patients on follow-up at the Jimma Medical Center (JMC), Jimma, Southwest Ethiopia.

Methods: A facility-based cross-sectional study involving 124 participants (each 62) of T2DM patients and controls was conducted from October to November 2020 using a structured questionnaire. 5 mL of venous blood was drawn to assess OF, complete blood count, and blood glucose levels. EOF was investigated using a series hypotonic solution of NaCl. The supernatant of the centrifuged sample was transferred to cuvette test tubes, and the hemolysis stage was read on a spectrophotometer. The collected data were coded and entered into Epi-data Version 3.1. The analysis was performed using SPSS Version 23.

Results: Compared with non-diabetic controls, patients with T2DM had significantly increased EOF. FBG >126mg/dl (AOR=7.741, 95% CI: 1.562-38.360), PPBG >200 mg/dl (AOR=7.576, 95% CI: 1.519-37.791), RDW (AOR=4.558, 95% CI: 1.136-18.284) were significantly associated with abnormal EOF. A statistically significant increase in total white blood cells and absolute neutrophil counts (P < 0.001) were observed in T2DM patients. From RBC indices, red blood cell distribution width (RDW) and mean corpuscular volume (MCV) were significantly increased in T2DM patients (P < 0.001).

Conclusion: This study suggests that EOF was greater in patients with T2DM than in non-diabetic controls and was determined by FBG, PPBG, and RDW. The study also demonstrated that hematological index alterations were higher in T2DM subjects than in non-diabetic controls.

背景:糖尿病(DM)是全球最普遍的非传染性疾病之一。据估计,2019 年全球约有 9.3% 的普通人群患有糖尿病。目的:本研究旨在评估红细胞渗透脆性(EOF)及其决定因素,并比较埃塞俄比亚西南部吉马市吉马医疗中心(JMC)随访的T2DM患者的血液学指标:2020 年 10 月至 11 月,采用结构化问卷调查法对 124 名 T2DM 患者和对照组参与者(每组 62 人)进行了一项以医疗机构为基础的横断面研究。抽取 5 mL 静脉血以评估 OF、全血细胞计数和血糖水平。使用一系列低渗的氯化钠溶液调查 EOF。离心样本的上清液转移到比色皿试管中,在分光光度计上读取溶血阶段。收集的数据经编码后输入 Epi-data 3.1 版。使用 SPSS 23.0 版进行分析:与非糖尿病对照组相比,T2DM 患者的 EOF 明显增加。FBG >126mg/dl (AOR=7.741, 95% CI: 1.562-38.360)、PPBG >200 mg/dl (AOR=7.576, 95% CI: 1.519-37.791)、RDW (AOR=4.558, 95% CI: 1.136-18.284)与EOF异常明显相关。据统计,T2DM 患者的白细胞总数和中性粒细胞绝对数明显增加(P < 0.001)。从红细胞指数来看,T2DM 患者的红细胞分布宽度(RDW)和平均血球容积(MCV)明显增加(P < 0.001):本研究表明,与非糖尿病对照组相比,T2DM 患者的 EOF 值更高,并由 FBG、PPBG 和 RDW 决定。研究还表明,T2DM 患者的血液指标变化高于非糖尿病对照组。
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引用次数: 0
Type 1 Gaucher's Disease. A Rare Genetic Lipid Metabolic Disorder Whose Diagnosis Was Concealed by Recurrent Malaria Infections in a 12-Year-Old Girl. 1型戈谢病。一种罕见的遗传性脂质代谢紊乱,12 岁女孩因反复感染疟疾而被掩盖了诊断。
IF 2 Q3 Medicine Pub Date : 2024-01-20 eCollection Date: 2024-01-01 DOI: 10.2147/JBM.S444296
Yekosani Mitala, Abraham Birungi, Branchard Mushabe, John Manzi, Brian Ssenkumba, Raymond Atwine, Siyadora Ankunda

Introduction: Gaucher disease is a rare autosomal recessive lysosomal storage disease with unknown prevalence in Africa and no record of the disease exists in Uganda.

Case presentation: We report a case of a 12-year-old female, the last born of 6 from a family with no known familial disease who presented with non-neuronopathic Gaucher disease and superimposed malaria. The disease was initially misdiagnosed as hyperreactive malarial splenomegaly but was subsequently confirmed by examination of the bone marrow smear and core. The disease was managed supportively and splenectomy was done due to worsening hematological parameters. She currently takes morphine for bone pains in addition to physiotherapy.

Conclusion: Always HMS is a common complication in malaria endemic areas, other causes of hepatosplenomegaly need to be excluded before the diagnosis is made. Diagnosis and treatment of patients with rare conditions like GD is still a challenge in developing countries. Although splenectomy is indicated in GD, it should only be done when it is absolutely necessary.

导言:戈谢病是一种罕见的常染色体隐性溶酶体储积病,在非洲的发病率不详,乌干达也没有该病的记录:我们报告了一例 12 岁女性的病例,她是家中 6 个孩子中的最后一个,没有已知的家族性疾病。该病最初被误诊为高反应性恶性脾肿大,但随后通过骨髓涂片和骨髓核检查得到确诊。对该病进行了支持性治疗,由于血液学指标恶化,进行了脾脏切除术。目前,除了物理治疗外,她还服用吗啡治疗骨痛:在疟疾流行地区,肝脾肿大是一种常见的并发症,在确诊前需要排除导致肝脾肿大的其他原因。在发展中国家,诊断和治疗 GD 等罕见疾病患者仍是一项挑战。尽管脾切除术适用于 GD,但只有在绝对必要的情况下才能进行。
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引用次数: 0
Assessment of Hematological Profiles and Prognostic Role of Hemogram-Derived Novel Markers for Diabetes Mellitus and Its Complications Among Type 2 Diabetes Mellitus Adult Patients Attending Bishoftu General Hospital, Central, Ethiopia: A Comparative Cross-Sectional Study. 埃塞俄比亚中部 Bishoftu 综合医院就诊的 2 型糖尿病成人患者的血液学特征评估以及血液图谱衍生的糖尿病及其并发症新型标记物的预后作用:一项横断面比较研究。
IF 2 Q3 Medicine Pub Date : 2023-12-28 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S435452
Dereje Abebe Regassa, Girum Tesfaye Kiya, Regassa Alemu Kebede, Woyesa Beyene

Background: Diabetes is a chronic metabolic syndrome that is a global public health problem. Studies have used hematological parameters and hemogram-derived markers as predictors of poor glycemic and microvascular complications status in diabetics. However, the tendency to use these parameters is not fully evaluated in our context, and the evidence is inadequate. This study aimed to assess the hematological profiles and prognostic role of hemogram-derived novel markers in diabetes mellitus and its complications among DM patients at Bishoftu General Hospital, Ethiopia.

Methods: A comparative cross-sectional study was conducted among 261 participants from June 15 to August 12, 2022. A systematic random sampling technique was used to select participants. Data were collected using structured questionnaires, physical measurements, checklists, and laboratory tests. Hematological parameters and fasting blood glucose levels were determined from blood using Sysmex-XN550 and Cobas C311 analyzers, respectively. Blood smear was used to check Hematology analyzer output, and to screen participants for malaria parasites. Collected data were entered into Epi-data 3.1 and exported to SPSS-25. Data were analyzed by Chi-square, Mann-Whitney U-test, Kruskal-Wallis test, Post hoc test, and ROC curve. A P-value <0.05 was considered statistically significant.

Results: Total WBC, neutrophils, Monocyte, NLR, MLR, MPVLR, and PLR were significantly higher in poor glycemic and complicated T2DM; meanwhile, measured RBC parameters, RBC indices values were significantly lower in poor glycemic and complicated T2DM. The NLR, MLR, MPVLR, PLR, and NLR, MLR, MPVLR, RPR values were identified as predictors of poor glycemic and complication status in diabetic patients, respectively.

Conclusion: Significant increment of some hematological parameters and hemogram-derived markers, and their role in predicting poor glycemic and microvascular complications were identified in diabetic patients. Routine screening of hematological parameters and use of hemogram-derived markers for monitoring of altered health status in DM is very important in the improvement of patient quality of life.

背景:糖尿病是一种慢性代谢综合征,是一个全球性的公共卫生问题。有研究将血液学参数和血液图衍生标记物作为糖尿病患者血糖和微血管并发症状况不佳的预测指标。然而,在我国,使用这些参数的倾向尚未得到充分评估,证据也不充分。本研究旨在评估埃塞俄比亚 Bishoftu 综合医院糖尿病及其并发症患者的血液学特征和血液图衍生新标记物的预后作用:方法:2022 年 6 月 15 日至 8 月 12 日,对 261 名参与者进行了横断面比较研究。研究采用系统随机抽样技术选取参与者。通过结构化问卷、体格测量、核对表和实验室测试收集数据。血液学参数和空腹血糖水平分别使用 Sysmex-XN550 和 Cobas C311 分析仪测定。血液涂片用于检查血液分析仪的输出结果,并筛查参与者体内的疟疾寄生虫。收集的数据输入 Epi-data 3.1,并导出到 SPSS-25。数据分析采用卡方检验(Chi-square)、曼-惠特尼U检验(Mann-Whitney U-test)、克鲁斯卡尔-瓦利斯检验(Kruskal-Wallis test)、事后检验(Post hoc test)和ROC曲线。A P值结果:白细胞总数、中性粒细胞、单核细胞、NLR、MLR、MPVLR和PLR在血糖不良和复杂T2DM患者中显著升高;同时,RBC参数、RBC指数值在血糖不良和复杂T2DM患者中显著降低。NLR、MLR、MPVLR、PLR 和 NLR、MLR、MPVLR、RPR 值分别被确定为糖尿病患者血糖不良和并发症状况的预测因子:结论:在糖尿病患者中发现了一些血液学参数和血液图衍生标记物的显著增量,以及它们在预测不良血糖和微血管并发症中的作用。对血液学参数进行常规筛查,并使用血液图衍生标记物监测糖尿病患者的健康状况变化,对提高患者的生活质量非常重要。
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引用次数: 0
Predictors of Intensive Care Admission Among Adult Patients with Sickle Cell Disease in Eastern Province of Saudi Arabia. 沙特阿拉伯东部省镰状细胞病成人患者接受重症监护的预测因素。
IF 2 Q3 Medicine Pub Date : 2023-12-27 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S435861
Mortadah Alsalman, Zaenb Alsalman, Hussain Abduljaleel Alkhalifa, Aman N Alfaraj, Ali Alkhalifah, Qasem Almulihi

Purpose: Sickle cell disease (SCD) comprises a complex group of hematologic disorders that are collectively the most common monogenic disorder and are associated with increased risk of intensive care unit admission (ICU). The purpose of this study is to investigate factors that predict admission of adult patients with SCD to the ICU.

Patients and methods: This was a cross-sectional study that enrolled adult patients with SCD from Saudi Arabia.

Results: A total of 107 patients with SCD, with a median age 31.9±12.1 years, were evaluated retrospectively. Regarding predictors of ICU admission, patients who indicated a history of blood transfusions were at 8.047-fold higher risk of ICU admission (OR=8.047; 95% CI=2.392-27.07; p=0.001). Patients who started hydroxyurea were at least 3.071 times more likely to be admitted than those who did not (OR=3.071; 95% CI=1.164-8.104; p=0.023). We also observed three or more hospitalizations per year to be associated with increased risk of ICU admission (OR=3.393; 95% CI=1.285-8.960; p=0.014), with those making 3 to 5 visits annually having at least 10.4 times higher risk (OR=10.38; 95% CI=10.098-98.19; p=0.041) and those with 6 to 10 ER admissions having 18 times higher risk (OR=18.00; 95% CI=2.149-150.8; p=0.008). Finally, patients with high WBC were predicted to have at least 3.34 times higher risk of ICU admission (OR=3.337; 95% CI=1.131-9.846; p=0.029).

Conclusion: SCD is a multi-systemic disease associated with increased morbidity and mortality. Recognition of high-risk features in patients helps to eliminate subjectivity in ICU referral decision. Frequent hospitalization and emergency visits, multiple blood transfusions, and elevated white blood cell count were significantly associated with a higher rate of ICU admission despite hydroxyurea usage.

目的:镰状细胞病(SCD)是一组复杂的血液病,是最常见的单基因疾病,与入住重症监护病房(ICU)的风险增加有关。本研究旨在调查预测 SCD 成年患者入住重症监护病房的因素:这是一项横断面研究,研究对象为沙特阿拉伯的成年 SCD 患者:回顾性评估了107名SCD患者,中位年龄为(31.9±12.1)岁。关于入住重症监护室的预测因素,表示有输血史的患者入住重症监护室的风险高出8.047倍(OR=8.047;95% CI=2.392-27.07;P=0.001)。开始使用羟基脲的患者比未使用羟基脲的患者入院风险至少高 3.071 倍(OR=3.071;95% CI=1.164-8.104;P=0.023)。我们还观察到,每年住院三次或三次以上的患者入住重症监护室的风险增加(OR=3.393;95% CI=1.285-8.960;p=0.014),每年就诊 3-5 次的患者风险至少增加 10.4 倍(OR=10.38;95% CI=10.098-98.19;p=0.041),急诊室就诊 6-10 次的患者风险增加 18 倍(OR=18.00;95% CI=2.149-150.8;p=0.008)。最后,预测白细胞高的患者入住 ICU 的风险至少高出 3.34 倍(OR=3.337;95% CI=1.131-9.846;P=0.029):结论:SCD是一种与发病率和死亡率增加相关的多系统疾病。识别患者的高危特征有助于消除重症监护室转诊决定的主观性。尽管使用了羟基脲,但频繁住院和急诊就诊、多次输血以及白细胞计数升高与较高的 ICU 入院率显著相关。
{"title":"Predictors of Intensive Care Admission Among Adult Patients with Sickle Cell Disease in Eastern Province of Saudi Arabia.","authors":"Mortadah Alsalman, Zaenb Alsalman, Hussain Abduljaleel Alkhalifa, Aman N Alfaraj, Ali Alkhalifah, Qasem Almulihi","doi":"10.2147/JBM.S435861","DOIUrl":"10.2147/JBM.S435861","url":null,"abstract":"<p><strong>Purpose: </strong>Sickle cell disease (SCD) comprises a complex group of hematologic disorders that are collectively the most common monogenic disorder and are associated with increased risk of intensive care unit admission (ICU). The purpose of this study is to investigate factors that predict admission of adult patients with SCD to the ICU.</p><p><strong>Patients and methods: </strong>This was a cross-sectional study that enrolled adult patients with SCD from Saudi Arabia.</p><p><strong>Results: </strong>A total of 107 patients with SCD, with a median age 31.9±12.1 years, were evaluated retrospectively. Regarding predictors of ICU admission, patients who indicated a history of blood transfusions were at 8.047-fold higher risk of ICU admission (OR=8.047; 95% CI=2.392-27.07; <i>p</i>=0.001). Patients who started hydroxyurea were at least 3.071 times more likely to be admitted than those who did not (OR=3.071; 95% CI=1.164-8.104; <i>p</i>=0.023). We also observed three or more hospitalizations per year to be associated with increased risk of ICU admission (OR=3.393; 95% CI=1.285-8.960; <i>p</i>=0.014), with those making 3 to 5 visits annually having at least 10.4 times higher risk (OR=10.38; 95% CI=10.098-98.19; <i>p</i>=0.041) and those with 6 to 10 ER admissions having 18 times higher risk (OR=18.00; 95% CI=2.149-150.8; <i>p</i>=0.008). Finally, patients with high WBC were predicted to have at least 3.34 times higher risk of ICU admission (OR=3.337; 95% CI=1.131-9.846; <i>p</i>=0.029).</p><p><strong>Conclusion: </strong>SCD is a multi-systemic disease associated with increased morbidity and mortality. Recognition of high-risk features in patients helps to eliminate subjectivity in ICU referral decision. Frequent hospitalization and emergency visits, multiple blood transfusions, and elevated white blood cell count were significantly associated with a higher rate of ICU admission despite hydroxyurea usage.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10757811/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139074147","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Magnitude of Red Blood Cell Alloimmunization Among Pregnant Women Attending Antenatal Care at Wolaita Sodo University Comprehensive Specialized Hospital, Southwest Ethiopia. 埃塞俄比亚西南部 Wolaita Sodo 大学综合专科医院接受产前检查的孕妇中红细胞同种免疫的程度。
IF 2 Q3 Medicine Pub Date : 2023-12-23 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S440952
Tamirat Ersino Kebamo, Abinet Tantu Kombe, Temesgen Eticha, Mesay Arkew, Getachew Nigussie Bolado, Tadele Lankrew Ayalew, Kassahun Haile, Getachew Alemu Walano

Background: Maternal red cell alloimmunization occurs when a woman's immune system becomes sensitive to unfamiliar red blood cell antigens. This leads to the production of alloantibodies, which can have serious implications for the fetus and newborn. However, there is a lack of comprehensive information about the extent of red cell alloimmunization in underdeveloped countries like Ethiopia. Therefore, this study aimed to determine the magnitude of red cell alloimmunization among pregnant women attending antenatal care at Wolaita Sodo University Comprehensive Specialized Hospital from September 01 to November 30, 2022.

Methods: In this institutional-based cross-sectional study, 422 pregnant women were participated and recruited using a systematic random sampling technique. Data on sociodemographic characteristics, obstetric history, and other clinical information were collected using structured questionnaires through face-to-face interview. Blood grouping and indirect antihuman globulin tests were performed. The relationship between red cell alloimmunization and the independent variables was determined using the chi-square test. P-value <0.05 was considered statistical significance.

Results: In this study, the blood group distributions among the participants were as follows: O, 177 (41.9%); A, 124 (29.4%); B, 76 (18%); and AB, 45 (3.86%). Among the pregnant women included in the study, a total of 51 (12.08%) were identified as RhD-negative. Out of these RhD-negative women, 5 (9.8%) were found to have developed alloimmunization with red blood cell antigens. Miscarriage and post-partum hemorrhage were found to be important factors associated with the occurrence of red cell alloimmunization in these women.

Conclusion: This study showed that one out of ten pregnant women was alloimmunized. Therefore, antenatal blood grouping and indirect antihuman globulin screening should be performed routinely to manage and minimize the undesirable outcomes of alloimmunization during pregnancy.

背景:当妇女的免疫系统对陌生的红细胞抗原变得敏感时,就会发生母体红细胞同种免疫。这会导致产生同种抗体,对胎儿和新生儿造成严重影响。然而,关于埃塞俄比亚等欠发达国家的红细胞同种免疫程度,目前还缺乏全面的信息。因此,本研究旨在确定 2022 年 9 月 1 日至 11 月 30 日期间在 Wolaita Sodo 大学综合专科医院接受产前检查的孕妇中红细胞同种免疫的程度:在这项以医院为基础的横断面研究中,采用系统随机抽样技术招募了422名孕妇。通过面对面访谈,使用结构化问卷收集社会人口学特征、产科病史和其他临床信息。此外,还进行了血型鉴定和间接抗人球蛋白试验。红细胞同种异体免疫与自变量之间的关系采用卡方检验。P 值结果:本研究中,参与者的血型分布如下:O型,177人(41.9%);A型,124人(29.4%);B型,76人(18%);AB型,45人(3.86%)。在参与研究的孕妇中,共有 51 人(12.08%)被确认为 RhD 阴性。在这些 RhD 阴性孕妇中,发现有 5 名(9.8%)孕妇出现了红细胞抗原同种免疫。流产和产后出血是这些妇女发生红细胞同种免疫的重要相关因素:这项研究表明,每 10 名孕妇中就有 1 名存在同种免疫。因此,应常规进行产前血型和间接抗人球蛋白筛查,以控制和尽量减少孕期同种免疫的不良后果。
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引用次数: 0
Current Status and Challenges in Delivering Comprehensive Care for Patients with Hemophilia. 为血友病患者提供全面护理的现状与挑战。
IF 2 Q3 Medicine Pub Date : 2023-12-15 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S446204
Shosaku Nomura

The importance of comprehensive care as a treatment strategy for patients with hemophilia is recognized worldwide. Comprehensive care entails addressing full spectrum of medical and psychological aspects impacting both patients and their families. The primary objective of comprehensive care for individuals with hemophilia is to enable them to lead their daily lives just as anyone else would. To achieve this goal, it is necessary to have a positive and collaborative approach across various healthcare disciplines. This extends beyond clinical specialists, encompassing pediatricians, hematologists, orthopedic surgeons, dental and oral surgeons, gynecologists, nurses, physical therapists, clinical psychologists, and other professionals from diverse fields. This review article discusses the current status and challenges associated with comprehensive care for patients with hemophilia. We categorize these challenges as follows: hemophilic arthritis, rehabilitation, oral care, transitioning from pediatric to adult care, addressing carrier issues, and providing psychological care. There is still substantial work to be undertaken in addressing these hurdles and advancing the quality of comprehensive care for hemophilia patients.

综合护理作为血友病患者的治疗策略,其重要性已得到世界公认。综合治疗需要全面解决影响患者及其家人的医疗和心理问题。为血友病患者提供全面护理的主要目的是让他们能够像其他人一样过日常生活。为了实现这一目标,有必要在各个医疗保健领域采取积极的合作方式。这不仅包括临床专家,还包括儿科医生、血液病医生、整形外科医生、牙科和口腔外科医生、妇科医生、护士、理疗师、临床心理学家以及其他来自不同领域的专业人士。这篇综述文章讨论了与血友病患者综合治疗相关的现状和挑战。我们将这些挑战归纳如下:血友病关节炎、康复、口腔护理、从儿科护理过渡到成人护理、解决携带者问题以及提供心理护理。要解决这些障碍并提高血友病患者的综合治疗质量,仍有大量工作要做。
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引用次数: 0
Hyperviscosity Syndrome Induced Bilateral Visual and Auditory Impairment in Therapy Resistant Waldenström Macroglobulinemia with MYD88 and CXCR4 Mutations. MYD88和CXCR4突变的治疗耐药的瓦尔登斯特伦巨球蛋白血症患者高粘滞性综合征引起的双侧视觉和听觉障碍
IF 2 Q3 Medicine Pub Date : 2023-12-15 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S424072
Marie M Plante, ErinMarie O Kimbrough, Amit K Agarwal, Liuyan Jiang, Kirk Bourgeois, Greta C Stamper, Michael W Stewart, Han W Tun

Hyperviscosity syndrome (HVS) is an emergent complication of Waldenström macroglobulinemia (WM) characterized by visual, neurologic, and rarely auditory impairment. We report a 69-year-old female with MYD88 and CXCR4-mutant WM who developed HVS resulting in bilateral blindness and deafness associated with neurologic manifestations including confusion, severe generalized weakness, and imbalance. Ophthalmologic evaluation revealed bilateral central retinal vein occlusion (CRVO), diffuse retinal hemorrhages, macular edema, and serous macular detachments (SMD). Magnetic resonance imaging of the brain showed bleeding in the inner ears. Management was challenging as her WM was resistant to systemic therapies including bendamustine + rituximab (BR) and rituximab + bortezomib + dexamethasone (RVD). Bruton's tyrosine kinase inhibitors could not be used initially due to ongoing lower gastrointestinal bleeding. She required five total sessions of plasma exchange and was finally initiated on zanubrutinib, achieving a partial response. She also received intravitreal bevacizumab with rapid resolution of the retinal hemorrhages but with little improvement of the SMD. She had partial restoration of her hearing in the right ear and only slight improvement in her bilateral visual deficits. The management of HVS in frail, elderly patients with therapy-resistant WM can be challenging. In these cases, plasma exchange is required until an effective systemic therapy can be safely instituted. Genomic profiling is important in the management of WM as it can predict treatment resistance and guide therapeutic decisions.

高粘滞性综合征(HVS)是瓦尔登斯特伦巨球蛋白血症(WM)的一种突发并发症,其特征是视觉、神经和听觉损伤,但很少发生。我们报告了一名患有 MYD88 和 CXCR4 突变 WM 的 69 岁女性患者,她患上了 HVS,导致双侧失明和耳聋,并伴有神经系统表现,包括意识模糊、严重全身无力和失衡。眼科评估显示,患者出现双侧视网膜中央静脉闭塞(CRVO)、弥漫性视网膜出血、黄斑水肿和浆液性黄斑脱离(SMD)。脑部磁共振成像显示内耳出血。由于她的WM对包括苯达莫司汀+利妥昔单抗(BR)和利妥昔单抗+硼替佐米+地塞米松(RVD)在内的全身疗法产生耐药性,因此治疗具有挑战性。由于持续的下消化道出血,布鲁顿最初无法使用酪氨酸激酶抑制剂。她总共需要进行五次血浆置换,最后开始使用扎鲁替尼,并取得了部分应答。她还接受了玻璃体内贝伐单抗治疗,视网膜出血很快得到缓解,但SMD改善甚微。她的右耳听力部分恢复,双侧视力障碍仅略有改善。对于体弱的老年 WM 患者,HVS 的治疗具有挑战性。在这些病例中,需要进行血浆置换,直到可以安全地进行有效的全身治疗。基因组图谱分析在 WM 的治疗中非常重要,因为它可以预测耐药性并指导治疗决策。
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引用次数: 0
Real-World Amount of Clotting Factor Concentrates Dispensed and Annual Medical Expenditures for Japanese Patients with Hemophilia B 日本 B 型血友病患者凝血因子浓缩剂的实际配量和年度医疗支出
IF 2 Q3 Medicine Pub Date : 2023-12-01 DOI: 10.2147/jbm.s418818
Katsuyuki Fukutake, K. Togo, Lin Xu, Leona Markson, José Maria Alvir, Ian Winburn, T. Karumori
Methods: This retrospective study comprised patients with hemophilia B (N=197) who had filled prescriptions for FIX concentrates reported in Japan’s Medical Data Vision database
研究方法:这项回顾性研究的对象是血友病 B 患者(N=197),他们开具的 FIX 浓缩液处方已在日本医疗数据视觉数据库中进行了报告。
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引用次数: 0
Paroxysmal Nocturnal Hemoglobinuria: Current Management, Unmet Needs, and Recommendations 阵发性夜间血红蛋白尿:当前管理、未满足的需求和建议
IF 2 Q3 Medicine Pub Date : 2023-12-01 DOI: 10.2147/JBM.S431493
Monika Oliver, C. Patriquin
Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-rare, acquired clonal abnormality, which renders hematopoietic cells exquisitely sensitive to complement-mediated destruction. Classical features of PNH include intravascular hemolytic anemia, increased thrombotic risk, and manifestations related to end-organ damage (eg fatigue, chest pain, dyspnea, renal failure, and pulmonary hypertension). With supportive care alone, mortality rate of patients with PNH is approximately 35%. The anti-C5 monoclonal antibody, eculizumab, was the first targeted therapy approved for PNH, and led to improved hemoglobin, quality of life, reduced transfusion need, reduced thrombosis, and greater overall survival. More recently, therapeutics such as longer acting anti-C5 (ravulizumab) and anti-C3 (pegcetacoplan) medications have been approved, along with other novel therapeutics in late-stage clinical trials. Biosimilars of eculizumab are also now available. Proximal inhibitors (against C3, factor B, and factor D) have shown significant improvements in hemoglobin and transfusion-avoidance in patients who remain anemic despite C5 inhibition. Despite these novel therapies, some unmet challenges remain, including management of breakthrough hemolysis, clinically significant iatrogenic extravascular hemolysis, optimal management in pregnancy, and infection risk mitigation as new targets in the complement system are blocked. In addition, the use of self-administered subcutaneous and oral therapies raises concerns around treatment adherence and the risks of uncontrolled terminal complement. Given the ultra-rare nature of PNH, development is underway of a centralized international registry to capture and analyze the data as they mature for various new therapies and characterize the clinical challenges related to PNH management.
阵发性夜间血红蛋白尿(PNH)是一种极其罕见的获得性克隆异常,它使造血细胞对补体介导的破坏非常敏感。PNH的典型特征包括血管内溶血性贫血、血栓形成风险增加和终末器官损害相关表现(如疲劳、胸痛、呼吸困难、肾功能衰竭和肺动脉高压)。仅通过支持性治疗,PNH患者的死亡率约为35%。抗c5单克隆抗体eculizumab是首个被批准用于PNH的靶向治疗药物,改善了血红蛋白,改善了生活质量,减少了输血需求,减少了血栓形成,提高了总生存率。最近,诸如长效抗c5 (ravulizumab)和抗c3 (pegcetacoplan)药物等治疗药物已被批准,以及其他处于后期临床试验的新型治疗药物。eculizumab的生物仿制药也已上市。近端抑制剂(针对C3、因子B和因子D)在C5抑制后仍然贫血的患者中显示出血红蛋白和输血避免的显著改善。尽管有这些新疗法,但仍存在一些未解决的挑战,包括突破性溶血的管理,临床显著的医源性血管外溶血,妊娠期的最佳管理,以及作为补体系统新靶点的感染风险降低。此外,使用自我给药的皮下和口服治疗引起了对治疗依从性和不受控制的末期补体风险的关注。鉴于PNH的超罕见性质,目前正在开发一个集中的国际注册表,以捕获和分析各种新疗法成熟的数据,并描述与PNH管理相关的临床挑战。
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引用次数: 0
Patient Blood Management and Its Role in Supporting Blood Supply. 患者血液管理及其在支持血液供应方面的作用。
IF 2 Q3 Medicine Pub Date : 2023-11-30 eCollection Date: 2023-01-01 DOI: 10.2147/JBM.S387322
Richard R Gammon, Rounak Dubey, Gaurav K Gupta, Colleen Hinrichsen, Aikaj Jindal, Divjot Singh Lamba, Sadhana Mangwana, Amita Radhakrishnan Nair, Shaughn Nalezinski, Christopher Bocquet

Blood donors and voluntary blood donations are essential for ensuring the blood supply that can be maintained by good patient blood management (PBM) practices. This review article explores the role of blood donation in PBM and highlights the importance of donor screening and selection processes in different regions worldwide. The donor health questionnaires and the focused physical examination guidelines have changed in the last decade to increase donor and recipient safety. This article also discusses the status of transfusion practices, including the challenges of ensuring a safe blood supply. Significant among these are the effects of the COVID-19 pandemic on the blood supply chain and the impact of an aging donor population, especially. Promoting autologous donations and other blood conservation strategies are suggested to mitigate these issues. The role of replacement donors and the upper age limit for voluntary blood donation may be decided based on the demography and donor pool. The involvement of C-suite executives is also critical in implementing and running a successful PBM program. The review highlights how these different aspects of blood donation are integral to a successful PBM program and the safety of patients who receive blood transfusions.

献血者和自愿献血对于确保血液供应至关重要,而良好的患者血液管理(PBM)实践可以维持血液供应。这篇综述文章探讨了献血在患者血液管理中的作用,并强调了全球不同地区献血者筛查和选择过程的重要性。在过去十年中,献血者健康调查问卷和重点身体检查指南都发生了变化,以提高献血者和受血者的安全。本文还讨论了输血实践的现状,包括确保血液供应安全所面临的挑战。其中最重要的是 COVID-19 大流行对血液供应链的影响,尤其是献血人群老龄化的影响。为缓解这些问题,建议推广自体捐献和其他血液保护策略。替代献血者的作用和自愿献血的年龄上限可根据人口结构和献血者库来决定。C-suite 高管的参与也是成功实施和运行 PBM 计划的关键。本综述强调了献血的这些不同方面对于成功的 PBM 计划和输血患者的安全是如何不可或缺的。
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引用次数: 0
期刊
Journal of Blood Medicine
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