Advances in Hematology最新文献

In Africa, sickle cell disease (SCD) is a major public health problem with over 200,000 babies born per year. In Ghana, approximately 15,000 (2%) of Ghanaian newborns are diagnosed with SCD annually. A retrospective review of medical records of all SCD patients aged 13 years and above, who presented to the sickle cell clinic at Ghana Institute of Clinical Genetics (GICG), Korle-Bu, from 1st January 2013 to 31st December 2014, was carried out, using a data abstraction instrument to document their phenotypes, demographics, attendance/clinic visits, pattern of attendance, and common complications seen. During the period under review 5,451 patients were seen at the GICG, with 20,788 clinic visits. The phenotypes were HbSS (55.7%) and HbSC (39.6%) with other sickle cell phenotypes (4.7%). Out of the 20,788 clinic visits, outpatient visits were 15,802 (76%), and urgent care visits were 4,986 (24%), out of which 128 (2.6%) patients were admitted to the Teaching Hospital for further management of their acute complications. There were 904 patient referrals (out of 5,451 patients) for specialist care; the 3 specialties that had the most referrals were Obstetrics and Gynaecology (168 patients), Orthopaedics (150 patients), and Ophthalmology (143 patients). In 2014, complications seen at KBTH included 53 patients with avascular necrosis (AVN) and 61 patients with chronic leg ulcers. Our centre has a large number of patients living with sickle cell disease. From our experience, early recognition and referral of sickle cell related complications can reduce morbidity and mortality associated with this disease. A multidisciplinary approach to care of SCD patients is therefore important.

In the field of medicine, the analysis of blood is one of the most important exams to determine the physiological state of a patient. In the analysis of the blood sample, an important process is the counting and classification of white blood cells, which is done manually, being an exhaustive, subjective, and error-prone activity due to the physical fatigue that generates the professional because it is a method that consumes long laxes of time. The purpose of the research was to develop a system to identify and classify blood cells, by the implementation of the networks of Gaussian radial base functions (RBFN) for the extraction of its nucleus and subsequently their classification through the morphological characteristics, its color, and the distance between objects. Finally, the results obtained with the validation through the coefficient of determination showed an overall accuracy of 97.9% in the classification of the white blood cells per individual, while the precision in the classification by type of cell evidenced results in 93.4% for lymphocytes, 97.37% for monocytes, 79.5% for neutrophils, 73.07% for eosinophils, and a 100% in basophils with respect to the professional. In this way, the proposed system becomes a reliable technological support that contributes to the improvement of the analysis for identification of blood cells and therefore would benefit the low-level hematology establishments as well as to the processes of research in the area of medicine.

Myelodysplastic syndromes (MDS) are a heterogeneous group of diseases characterized by ineffective hematopoiesis and a wide spectrum of manifestations ranging from indolent and asymptomatic cytopenias to acute myeloid leukemia (AML). MDS result from genetic and epigenetic derangements in clonal cells and their surrounding microenvironments. Studies have shown associations between MDS and other autoimmune diseases. Several immune mechanisms have been identified in MDS, suggesting that immune dysregulation might be at least partially implicated in its pathogenesis. This has led to rigorous investigations on the role of immunomodulatory drugs as potential treatment options. Epigenetic modification via immune check point inhibition, while well established as a treatment method for advanced solid tumors, is a new approach being considered in hematologic malignancies including high risk MDS. Several trials are looking at the efficacy of these agents in MDS, as frontline therapy and in relapse, both as monotherapy and in combination with other drugs. In this review, we explore the utility of immune checkpoint inhibitors in MDS and current research evaluating their efficacy.

We have investigated the frequencies of regulatory T cells and the level of FOXP3 isoforms expression in peripheral blood of patients with myelodysplastic syndromes and found the significant reduction of regulatory T cells at all stages of the disease. At the same time in untreated patients, we observed the shift in the FOXP3 isoforms expression profile towards the full-length molecule possibly due to inflammation. Based on the already known information about the potentially higher functional activity of FOXP3 molecule lacking exon 2, we have also hypothesized that our finding may explain the high risk of autoimmune disorders in this disease.

Lower levels of vitamin D have been documented in many patients with sickle cell disease (SCD), but data are still inconclusive regarding the association between vitamin D deficiency (VDD) and the occurrence or the severity of various SCD complications. Our study aimed to detect the prevalence of vitamin D deficiency among Egyptian patients with SCD and to associate it with the clinical course of the disease. We measured the level of 25-hydroxy vitamin D in 140 children (age from 4.3 to 15.5years), 80 patients with SCD and 60 controls using enzyme-linked immunosorbent assay. Vitamin D was deficient in 60% of SCD compared to 26.7% of controls. Severe VDD was significantly higher in SCD patients than controls. Patients were divided into 2 groups; Normal group (32 patients) and Deficient group (48 patients). There were statistically significant differences between the 2 groups regarding their age, height percentile, the presence of clinical jaundice, and osseous changes (P values 0.043, 0.024, 0.001, and 0.015, respectively). Hemoglobin and hematocrit values were significantly lower in Deficient group (P values 0.022 and 0.004, respectively) while the levels of aspartate aminotransferase, lactate dehydrogenase, and total and indirect bilirubin were significantly higher in the same group (P values 0.006, 0.001, 0.038, and 0.016, respectively). The frequency of blood transfusions, hospitalization, and vasoocclusive crisis previous year as well as the history of bone fracture and recurrent infections proved to be significantly higher in Deficient group. These findings suggest that VDD may play a role in the pathogenesis of hemolysis and other complication of SCD. Vitamin D monitoring and supplementation in patients with SCD should be implemented as a standard of care to potentially improve health outcomes in these affected patients.

Background: In pregnancy, anemia is an important factor associated with an increased risk of maternal, fetal, and neonatal mortality, poor pregnancy outcomes, and impaired cognitive development, particularly in developing countries like Ethiopia. This study aimed to assess prevalence and factors associated with anemia among pregnant women attending antenatal clinic at St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia.

Method: A cross-sectional health facility based study was conducted on 284 pregnant women to assess prevalence and factors associated with anemia at St. Paul's Hospital Millennium Medical College from June to August 2014. Data on sociodemographic and clinical characteristics of the study participants were collected using a pretested structured questionnaire by interview and review of medical records. About 4 ml of venous blood was collected from each subject for peripheral blood film and complete blood counts (CBC). Binary Logistic regression analysis had been used to check for association between dependent and independent variables. In all cases, P value less than 0.05 was considered statistically significant.

Result: The prevalence of anemia was found to be 11.6% (95 % CI; 7.8%-14.8%). Pregnant women in the second [AOR (95% CI), 6.72 (1.17-38.45), and P=0.03] and third trimester [AOR (95% CI), 8.31 (1.24-55.45), and P=0.029] were more likely to be anemic when compared to pregnant women in their first trimester. Pregnant women who did not receive iron/folic acid supplementation [AOR (95%CI), 4.03(1.49-10.92), and P=0.01] were more likely to be anemic when compared to pregnant women who did take supplementations.

Conclusion: In this study the prevalence of anemia in pregnancy was low compared to the findings of others. Gestational age (trimester) and iron/folic acid supplementation were statistically associated with anemia. Therefore, iron supplementation and health education to create awareness about the importance of early booking for antenatal care are recommended to reduce anemia.

Background: Automation helps improve laboratory operational efficiency and reduce the turnaround time. Pneumatic tube systems (PTS) automate specimen transport between the lab and other areas of the hospital. Its effect on complete blood count (CBC) and coagulation is still controversial.

Aim: To study the effects of pneumatic tube system sample transport on complete blood count and coagulation parameters to compare them with hand delivered samples.

Methods: 75 paired samples for complete blood count and 25 paired samples for coagulation analysis were compared between samples sent via pneumatic tube system and hand delivered system.

Results: PTS showed significant decrease in red cell indices such as MCV and RDW and increase in MCHC. Other red cell parameters and WBC parameters showed no statistical significant difference. Statistically significant increase in platelet count was observed with PTS samples. However, these differences were clinically insignificant. No significant effect of PTS was found in PT and APTT samples compared to the hand delivered samples.

Conclusion: Despite statistically significant changes in RBC parameters such as MCV, RDW, and MCHC and platelet count, these changes were clinically insignificant. Hence, blood samples for CBC and coagulation assay can safely be transported via our hospital's PTS. However, further studies on platelet count are warranted to ensure safe transport and accuracy of the results.

Background: Transfusion of red blood cells (RBC) is an essential therapeutic tool in sickle cell disease (SCD). Repeated RBC transfusions can cause alloimmunization which causes difficulty in cross-matching and finding compatible blood for transfusions. This study aimed to investigate the frequency of RBC alloimmunization and related risk factors among Palestinian SCD patients.

Materials and methods: A multicenter cross-sectional study on 116 previously transfused SCD patients from three centers in West Bank, Palestine. Demographic, medical data and history of transfusion were recorded. Blood samples were collected from transfused consenting SCD patients. Gel card method was used for antibody screening and identification. In all patients, autocontrol and direct antiglobulin (DAT) test were performed using polyspecific (anti-IgG + C3d) anti-human globulin (AHG) gel cards for the detection of autoantibodies.

Results: Of the SCD patients, 62 (53.4%) patients were HbSS and 54 (46.6%) patients were sickle β-thalassemia (S/β-thal). There were 53 (45.7%) females and 63 (54.3%) males. Mean age was 18.8 years (range 3-53 years). The frequency of RBC alloimmunization among SCD patients was 7.76%, with anti-K showing the highest frequency (33.3%) followed by anti-E (22.2%), anti-D (11.1%), anti-C (11.1%), and anti-c (11.1%). All reported IgG alloantibodies were directed against antigens in the Rh (66.7%) and Kell (33.3%) systems. Older ages of patients, increased number of blood units transfused, and splenectomy were the commonest risk factors for alloimmunization in our study.

Conclusions: RBC alloimmunization rate among Palestinian SCD patients is low compared to neighboring countries and countries all over the world but still warrants more attention. Phenotyping of donors/recipients' RBC for Rh antigens and K₁ (partial phenotype matching) before their first transfusion may reduce the incidence of alloimmunization.

Introduction: Inflammation is widely recognized to play an important role in cancer progression and is related to thrombosis. Soluble P-selectin (sP-selectin) is one of several biomarkers that may be predictive of thrombosis in cancer. This study aimed to investigate the correlation between monocyte count and sP-selectin in various stages of nasopharyngeal carcinoma.

Methods: Fifty-five patients with nasopharyngeal carcinoma were divided into three groups according to nodal and distant metastasis (group of stages I-IVA, IVB, and IVC). Monocyte count was calculated from routine peripheral blood examination, while sP-selectin level was measured using commercial ELISA kit.

Results: The monocyte count of subjects in groups IVB and IVC was significantly higher compared to group I-IVA (707/μL versus 528/μL, p = 0.022; 841/μL versus 528/μL, p = 0.005). Plasma levels of sP-selectin in group IVC were higher than group I-IVA (59.5 ng/mL versus 41.97 ng/mL, p = 0.001) and group IVB (59.5 ng/mL versus 45.53 ng/mL, p = 0.007). In subjects with high monocyte count (>665/μL), there was moderate correlation between monocyte count and sP-selectin (r = 0.436, p = 0.022).

Conclusion: Advanced stages of nasopharyngeal carcinoma had higher levels of monocyte count and sP-selectin compared to earlier stages. Monocyte count was correlated with sP-selectin especially in high monocyte count subgroup.