Journal of Blood Medicine最新文献

Background: Recently, an increasing number of studies have suggested dual-specificity phosphatase 23 (DUSP23) is a critical factor in the development of diffuse connective tissue disease and may be a valuable biomarker for primary human cancers. However, there is a lack of comprehensive studies on the prognostic significance of DUSP23 expression in acute myeloid leukemia (AML).

Methods: RNA sequencing data from The Cancer Genome Atlas (TCGA) (AML = 173), Genotype-Tissue Expression (GTEx) (healthy controls = 70) and GEO (AML = 461, healthy controls = 76) databases were used to compare DUSP23 expression between AML patients and healthy controls. The overall survival (OS) of DUSP23 in AML was evaluated using Kaplan-Meier Cox regression. Furthermore, univariate Cox regression and multivariate Cox regression analysis were used to determine whether DUSP23 was an independent prognostic factor for AML. We then verified the expression level and prognostic significance of DUSP23 in our cohort (AML = 128, healthy controls = 31). In addition, functional enrichment analysis of DUSP23-related DEGs was performed through gene set enrichment analysis (GSEA) and protein-protein interaction (PPI) network analysis.

Results: The expression level of DUSP23 is significantly higher in AML patients than in healthy controls in TCGA, GTEx, GEO databases and our cohort. By multivariate analysis, high expression of DUSP23 is a poor prognostic indicator of OS in the TCGA database. Next, we verified the role of DUSP23 as an adverse prognostic biomarker in our cohort. Enrichment analysis of related genes showed that DUSP23 may regulate important signal pathways in hematological tumors including the MAPK pathways. It is suggested by the PPI network that DUSP23, along with IMP3, MRPL4, MRPS12, POLR2L, and ATP5F1D may play a role in the process of AML.

Conclusion: The study demonstrated high expression of DUSP23 could serve as a poor independent prognostic biomarker in AML.

Background: Human exposure to benzene is associated with many adverse health effects. It is mainly related to impairment of the hematopoietic system and bone marrow suppression, causing abnormalities in hematological parameters. However, the reports obtained from different studies are contradictory, and there are little data regarding the hematological parameters of gas station workers in the study area. Therefore, this study aimed to evaluate the hematological parameters of gas station workers in Hosanna town, southwest Ethiopia, from May 01 to June 15, 2020.

Methods: A comparative cross-sectional study was conducted by involving 180 (60 gas-stations workers and 120 controls) participants. Socio-demographic and related data of the study participants were collected using a pre-tested structured questionnaire through face-to-face-interviews. All phases of quality assurance were maintained, and hematological parameters were determined using Uni-Cel DxH 800 automated hematological analyzer. Independent sample T-test, Mann-Whitney U-test, and one-way ANOVA were used for data analysis. Statistical significance was declared at P<0.05.

Results: Statistically significant difference was observed in hematological parameters of gasoline-workers and control groups. The mean of red blood cell count among gasoline-workers was significantly reduced as compared to control groups (p=0.007). In addition, the median of hemoglobin levels among gasoline-workers was significantly decreased as compared to the control groups (p=0.001). In contrast, a significant increase was observed in median of absolute eosinophil count among the gasoline-workers as compared to control groups (p=0.01). The mean of mean cell volume was significantly decreased with respect to the duration of work experience (p=0.04).

Conclusion: In this study, a statistically significant difference was observed in some hematological parameters of gas station workers compared to the control group. Therefore, medical observation and periodic medical check-ups of the hematological profile should be considered to prevent the development of medical complications.

Brentuximab vedotin (BV) and nivolumab are increasingly utilized as a novel regimen in patients with relapsed/refractory classical Hodgkin lymphoma (cHL). A 26-year-old male presented to the hospital with refractory diabetic ketoacidosis and multiple electrolyte abnormalities, 9 days after the first dose of brentuximab vedotin and nivolumab for recurrent classical Hodgkin lymphoma. During his hospitalization, he developed multi-organ failure. His workup showed elevated cytokine levels concerning severe cytokine release syndrome (CRS) and hemophagocytic lymphohistiocytosis (HLH)-like syndrome. Despite treatment with CRS- and HLH-directed therapies, his clinical status deteriorated due to ongoing multifactorial shock and worsening multi-organ dysfunction, and comfort care measures were eventually pursued. To our knowledge, there have been no other cases reported of HLH-like syndrome after the combination of BV and nivolumab in patients with cHL. This case of a fatal adverse event following one dose of BV and nivolumab underscores the vital need for close monitoring of patients receiving this treatment regimen.

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.

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.

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.

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.

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.

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.

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.