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Background Apoptotic microparticles are responsible for almost all tissue factor activity of the plaque lipid core. We hypothesized that elevated levels of procoagulant microparticles could also circulate in the peripheral blood of patients with recent clinical signs of plaque disruption and thrombosis. The present study included 60 acute coronary syndrome (ACS) adult patients. Group I included 30 patients with diabetes mellitus who presented with ACS. Group II included 30 nondiabetic patients complaining of ACS and 25 healthy individuals as controls. ACS patients were further classified according to laboratory and radiological findings (troponin test and ECG) as follows: group A included 16 ST-segment elevation myocardial infarction (STEMI) patients, group B included 19 non-ST-segment elevation myocardial infarction (NSTEMI) patients, and group C included 25 patients with unstable angina. Traditional laboratory investigations and special laboratory assessments of CD144 fluorescein isothiocyanate by flow cytometry were performed. Results The present study found highly elevated CD144 percentages in diabetic ACS patients compared with healthy controls (P≤0.0001(, and highly elevated creatine kinase-MB (CK-MB), fasting sugar, total cholesterol, triglyceride, HDL, and LDL (P = 0.0001, 0.0001, 0.0002, 0.0002, 0.0001, and 0.0001, respectively). In contrast, nondiabetic ACS patients had significantly elevated CD144, CK-MB, total cholesterol, triglyceride, HDL, and LDL (P = 0.0001, 0.0001, 0.0001, 0.0021, 0.0001, and 0.0021, respectively), whereas fasting sugar and HbA1c did not change significantly. However, the patients in group B (NSTEMI) had significantly elevated CD144% in comparison with patients with unstable angina (group C) ( P = 0.05), but patients with group A (STEMI) had significantly elevated CK-MB compared with patients with unstable angina (group C) (P = 0.02). Conclusion The high levels of circulating microparticles of endothelial origin are increased in diabetic patients with coronary artery disease, suggesting an important role for endothelial injury in the prediction of ACS. Hyperglycemia in ACS is associated with enhanced local thrombin generation and platelet activation, as well as unfavorably altered clot features in patients with and without a previous history of diabetes.

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The initial hemostatic plug is not sufficient to prevent blood loss unless it is stabilized by the action of plasma factor XIII. Congenital or acquired factor XIII deficiency must be considered when a patient has a major bleeding disorder and all of the initial screening laboratory tests are normal, including prothrombin time, activated partial thromboplastin time, platelet count, and bleeding time. Here we report the case of a child with congenital factor XIII deficiency, who presented with bleeding from umbilical stump and spontaneous hematoma in the left buttock.

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Background Thalassemia is associated with the generation of labile iron in the red blood cells, which promotes the formation of reactive oxygen species, leading to cumulative cell damage. Ischemia modified albumin (IMA) is now suggested to reflect generalized oxidative stress. Objectives The aim of this study was to evaluate IMA in children with transfusion-dependent (TD) β-thalassemia and its relation with serum ferritin and iron chelation therapies. Patients and methods A total of 60 children with TD thalassemia were divided into three groups on the basis of the type of iron chelation therapy received: group A received oral deferiprone (DFP), group B received effervescent deferasirox (DFX), and group C did not receive any type of iron chelation therapy. A total of 20 age-matched and sex-matched healthy children were included as controls. Serum ferritin and IMA were determined for all participants. Results There were significant increases in serum ferritin and IMA levels in thalassemic children than in controls (P < 0.001 for each). Children on DFP and DFX had significantly lower IMA levels compared with children not receiving any iron chelation therapy (P < 0.001 and 0.01, respectively). Serum ferritin had a positive significant association with IMA (r = +0.27 and P = 0.03). Conclusion IMA was higher in children with TD thalassemia than in controls. Moreover, its level in thalassemic children on either DFP or DFX was significantly lower than its level in children not receiving any chelation therapy. Therefore, IMA may be a possible marker of iron-induced oxidative stress in β-thalassemia.

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Introduction The anaplastic lymphoma kinase (ALK) gene has been identified as a major neuroblastoma (NB) predisposition gene. Furthermore, there are controversies on the correlation between ALK gene aberration and clinical outcome in neuroblastoma (NBL). Materials and methods We evaluated N-MYC/ALK gene copy number by fluorescence in situ hybridization and analyzed 32 bone marrow samples infiltrated by NB and analyzed their association with the clinical outcome of the patients. Results Although an increase in ALK gene aberration is a recurrent genetic abnormality of NB (50%, 16/32), ALK amplification was only present in one NB (3.2%, 1/32). In addition, ALK positivity also significantly correlates with N-MYC gene copy number (P < 0.000). Kaplan-Meier survival analysis indicated that the N-MYC/ALK aberration is correlated with decreased overall survival (OS) in NB. A better prognosis was observed in patients who were negative for N-MYC/ALK normal compared with those who were positive for N-MYC/ALK aberration tumors. Furthermore, ALK aberrations were significantly correlated with inferior survival in NB (P < 0.000). Conclusion ALK aberrations in NB were correlated with advanced tumor types and an increase in both N-MYC/ALK gene aberrations. ALK aberrations predict an inferior prognosis, which can be used as a prognostic factor in NB in clinical practice.

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Introduction Anemia is an almost constant complication of advanced renal failure, which may worsen pre-existing heart disease and, as a consequence, accelerate the progression of renal dysfunction, and patients with hepatitis C virus (HCV) infection are associated with higher hemoglobin and hematocrit values compared with those without the infection. Aim of the work The aim of this study was to evaluate iron status and erythropoiesis in patients with chronic hepatitis C infections who were on hemodialysis and to determine whether HCV infection would affect the iron status and erythropoiesis in such patients, through assessment of serum visfatin and prohepcidin. Patients and methods All patients included in the study were subjected to the following: full medical history and clinical examination; routine laboratory investigations; serum ferritin and serum iron evaluation; total iron-binding capacity with transferrin saturation; evaluation of serum visfatin level using an enzyme immunoassay; and determination of serum prohepcidin concentration using available enzyme-linked immunosorbent assay kit. Results A total of 107 chronic renal failure patients on hemodialysis (61 male and 46 female patients) were included in the study, and their ages ranged from 46 to 54 years, with a mean age of 50.5 ± 12.7. Of them, 61 were infected with HCV, and there was no significant relation with different causes of renal failure. Hemoglobin and hematocrit values were significantly higher in hemodialysis patients with HCV infection compared with those without HCV infection, with significant reduction of total iron supply and erythropoietin dosage/month. There were significant negative correlations between hemogloblin and hematocrit levels and iron supply and erythropoietin dosage/week, whereas significant positive correlation was observed between hemogloblin, hematocrit, serum iron, ferritin, transferrin saturation, as well as liver enzymes (aspartate transaminase, alanine transaminase) with serum visfatin and prohepcidin. Moreover, transferrin saturation, ferritin, and prohepcidin contributed independently to visfatin variance. Conclusion Serum levels of visfatin and prohepcidin are higher in chronic renal failure patients on hemodialysis and correlates with chronic hepatitis C infection, which is associated with an increased erythropoiesis, leading to lowering of the necessary erythropoietin dose and iron therapy.

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Background Red blood cell (RBC) alloimmunization is a major challenge to repeated transfusions in β-thalassemia-major patients. The aim of this study was to evaluate the magnitude of RBC alloimmunization and autoimmunization in regularly transfused Egyptian patients with β-thalassemia major and analyze factors that may be responsible for the development of antibodies. Patients and methods This study was conducted on 200 Egyptian β-thalassemia-major patients with age less than 16 years, who routinely visited the Pediatric Hematology Clinic of Ain Shams University Hospital for regular transfusions. All the patients underwent antibody screening. Patients with a positive antibody screen were further tested for antibody identification. The data were analyzed to find out the frequency of alloimmunization, and the patients' records were revised to analyze the factors influencing it. In case of pan-positivity of the antibody-screening cells and the autocontrol, adsorption by autogenic RBCs was performed to uncover the presence of alloantibodies. Results RBC alloantibodies were found in 21 (10.5%) patients. The most frequent alloantibodies encountered were anti-Kell (52.4%) and anti-E (19%). Autoantibodies were encountered in only one patient. They were the warm autoantibody type, and adsorbtion using autogenic RBCs was succesful in eliminating them. The frequency of blood transfusion, the transfusion index, serum ferritin, and the age at first transfusion showed a statistically significant correlation with alloimmunization ( P < 0.05). In contrast, there was no statistically significant association between the patients' sex, age, the ABO, Rh blood groups, and the spleen state and alloimmunization (P > 0.05). Conclusion We conclude that alloimmunization to RBC antigens is a relatively frequent finding among Egyptian transfusion-dependent thalassemic patients. The most frequent antibodies detected were against the Kell and Rh blood groups, mainly anti-Kell and anti-E. The majority of alloantibodies detected in this study were clinically significant.

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Introduction Lymphoma is considered the most common hematologic malignancy in Egypt and accounts for about 8.4% of all new cancer cases annually; however, the treatment outcome needs to be evaluated and compared with those achieved with the standards used worldwide. Patients and methods A retrospective pilot descriptive study of the outcome of patients with lymphoma treated at the Clinical Hematology and Bone Marrow Transplantation Units in Ain Shams University Hospitals over 2 years (2008 and 2009) was carried out. The study included 74 patients, 31 of whom were diagnosed with Hodgkin's lymphoma (HL) (group I), 10 with indolent non-Hodgkin lymphomas (NHL) (subgroup IIa), and 33 with aggressive NHL (subgroup IIb). Results In this study, NHL was the most common type, found in 58% of the cases, and was more common in older patients; low-grade lymphoma was more common in women and aggressive lymphoma was more common in men. However, HL was found in 42% of cases and was more common in young males. Hepatitis C virus was the most common associated infection; it was detected in 6% of HL and 38% of NHL patients, who were usually in advanced stage disease (80.64% stage III and IV vs. 19.36% stage I and II in HL and 89.19 vs. 10.81% in NHL patients). IPS Hodgkin lymphoma (HL) and International Prognostic Index (IPI) non Hodgkin lymphoma (NHL) were not predictors for treatment response or subsequent relapse. The best results of chemotherapy were achieved using ABVD with IFRT in patients with HL, FC protocol in low-grade NHL, and CHOP-R in high-grade NHL. An overall higher response rate was found in patients with HL compared with NHL patients. ASCT in relapsed patients at the time of the second complete remission did not lead to a significant improvement in disease-free survival or overall survival. Conclusion Although auto-SCT in NHL in our unit was not statistically significant, but it yielded a good result compared with the international one.

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Introduction b-Thalassemia major (B-TM) is a serious health problem in which children are in need of regular blood transfusions from a very young age to survive. They also need to receive iron chelation therapy to remove excess iron from their bodies, which imposes serious risk on their health and quality of life (QOL). Hence, this study was designed to assess the QOL of Egyptian B-TM children and adolescents in comparison with their healthy peers. Materials and methods A total of 127 B-TM patients and 65 healthy volunteers were enrolled and interviewed at the Ain Shams University Thalassemia Center. QOL assessment was made using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scale. Results The controls had higher QOL scores in all domains at the start of the study (P<0.0001). Compliant patients had higher total QOL scores (P = 0.004). High pretransfusion hemoglobin levels and low serum ferritin levels were independent predictors of better QOL scores. Conclusion B-TM patients had a poor QOL; high hemoglobin level and low iron overload were associated with improved QOL scores. Egyptian J Haematol 39:-0 © 2014 The Egyptian Society of Haematology.

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Introduction Fas is expressed on a majority of human leukemic cells. Fas-mediated cell death is involved in drug-induced apoptosis in various cell types. Hence, failure of apoptosis could lead to chemoresistance and may therefore have an impact on clinical outcome. The aim of the present study was to evaluate the percentage of Fas receptor expression on blast cells in patients with adult acute leukemia and blastic transformation phase of chronic myeloid leukemia (CML-BT), and to find out the impact of Fas expression on prognosis. Subjects and methods The participants of this study were 80 adult acute leukemia patients classified as follows: 40 acute myeloid leukemia (AML) patients, 32 ALL patients, and eight CML-BT patients. In addition, 10 age-matched and sex-matched healthy controls were also included in the study. Patients with acute leukemia were studied at diagnosis and after treatment. The diagnosis of AML, ALL, and CML-BT was assessed by morphological study, cytochemical analysis, and immunophenotyping of peripheral blood (PB) and bone marrow aspirate according to FAB classification. Fas expression on blast cells from bone marrow aspirate or PB samples of the patients or on normal monocytes, granulocytes, and lymphocytes obtained from PB samples of controls was measured using flow cytometry. The correlation between prognostic markers (age, sex, total leukocytic count, serum lactate dehydrogenase (LDH), and cytogenetic risk categories) and Fas expression levels on blast cells of leukemic patients at diagnosis was ascertained. After treatment, patients were followed up for periods ranging from 25 to 31 months. Results Fas expression was seen to be the highest on control monocytes (31.2 ± 6.95%), followed by granulocytes (24.8 ± 7.61%), whereas lymphocytes expressed the lowest levels (17.1 ± 4.01%) with a highly statistically significant difference (P < 0.001) between variables. The mean value of Fas expression by blast cells from AML patients at diagnosis was 41.72 ± 10.3%. AML patients were divided into the Fas-positive group [29 patients (72.5%)] and the Fas-negative group [11 patients (27.5%)]. The mean values of expression increased significantly from M1 to M5 with the weakest expression in M1 (20.28 ± 5.3%) and the strongest in M5 (52.91 ± 11.3%) with highly significant differences (P < 0.001) between Fas expression levels in different FAB subtypes of AML. The mean value of Fas expression in ALL patients was 42.87 ± 11.5%. Twenty-three (71.88%) patients were positive for Fas expression, whereas nine (28.12%) were negative. Fas expression was positive in 12/19 (63.2%) precursor B-ALL patients and in 11/13 (84.6%) T-ALL patients. The mean value of Fas expression was significantly higher (P = 0.039) in T-ALL (55.15 ± 7.8%) in comparison with precursor B-ALL (34.47 ± 5.76%). CML-BT patients were all transformed to AML type and expressed Fas with a mean of 31.0 ± 7.63%. Only three of them (37.5%) were Fas negative and the remaining five (62.5%) were Fas positive. The percentage of Fas-positive patients was 0% (0/1) in M1, 50% (2/4) in M2, 100% (1/1) in M4, and 100% (2/2) in M5. No significant differences could be detected between Fas-positive and Fas-negative patients with respect to baseline patient characters (age, sex, total leukocytic count, serum LDH levels, and cytogenetic risk category). Highly significant correlation was found between Fas-positive expression and response to therapy (P < 0.001) in AML and ALL patients. Fas-negative patients in CML-BT were nonresponders to chemotherapy with a significant correlation between low Fas expression and failure to respond to therapy (P = 0.049). Overall survival between Fas-positive and Fas-negative patients was statistically significant in AML, ALL, and CML-BT patients (P = 0.001, 0.002, and 0.002, respectively). Conclusion The mean value of Fas expression by blast cells from AML, ALL, and CML-BT patients at diagnosis was 41.72 ± 10.3, 42.87 ± 11.5, and 31 ± 7.63%, respectively. We can conclude that Fas receptor expression on blast cells from ALL, AML, and CML-BT patients could serve as an independent prognostic factor and help in monitoring the outcome of chemotherapy.

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Introduction Idiopathic thrombocytopenic purpura (ITP) is an autoimmune bleeding disorder that occurs because of enhanced peripheral platelet destruction. Antibodies and T cells are involved in the pathogenesis of the disease and, like other autoimmune diseases, patients with ITP have a peripheral deficiency in regulatory T cells (Treg) numbers and function that may be responsible for loss of tolerance. Our aim was to measure Tregs (CD4 ⁺ CD25 ^+high FoxP ⁺ 3) and levels of interleukins (IL-10 and IL-12) in peripheral blood mononuclear cell (PBMC) cultures from patients with ITP and analyze their relationship with the clinical features and outcome of treatment of ITP. Participants and methods Forty-five participants were included in this study, divided into two groups. Group I included 15 healthy children as a control group. Group II included 30 pediatric patients with ITP. According to treatment, group II was divided into three subgroups: group IIa (no treatment) included two (6.7%) patients, group IIb (steroid treatment) included 10 (33.3%) patients, and group IIc (steroid+intravenous immunoglobulin treatment) included 18 (60%) patients. ITP is diagnosed by platelet count less than 100 Χ 103/μl. Tregs were analyzed by flow cytometry. IL-10 and IL-12 in the supernatants of basal and lipopolysaccharide-stimulated PBMC cultures were estimated using an enzyme-linked immunosorbent assay. Results A significantly lower percentage of Tregs was found in patients than in controls (1.46 ± 0.97 vs. 7.09 ± 1.5%) and the lowest percentage of Tregs was recorded in group IIc. A positive correlation was observed between Tregs% and platelet count in the patient group. PBMCs from patients had significantly higher basal levels of IL-10 and IL-12, with a marked reduction in responsiveness to lipopolysaccharide in vitro compared with the controls. Conclusion Children with ITP had reduced Tregs% and IL-10/IL-12 imbalance. Thus, Tregs may play a role in modifying immune responses in these patients, resulting in new strategies of treatment and monitoring of disease activity.

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Background In the majority of cases, flow cytometry enables the differentiation of chronic lymphocytic leukemia (CLL) from mantle cell lymphoma (MCL). However, the diagnosis of CLL becomes challenging when CD23 is not expressed by the leukemic cells or in cases of MCL expressing CD23. CD200 is a membrane glycoprotein expressed on a subset of T and B lymphocytes. Its expression has been observed on human myeloma, plasma cells, and CLL cells. Aim of the work We investigated the pattern of expression of CD200 in CLL and MCL patients, aiming to clarify its possible role in differentiating these often overlapping disorders. Patients and methods This study was carried out on 30 patients with newly diagnosed CLL and 10 patients with MCL. Flow cytometric immunophenotyping was performed using the CD200 monoclonal antibody in addition to the standard panel of chronic lymphoproliferative diagnosis. Results CD200 was expressed in 100% of CLL cases with moderate intensity compared with only 10% of MCL cases with low intensity. Conclusion CD200 is a powerful addition to the routine flow cytometric panel in the differentiation between CLL and MCL with high sensitivity and specificity.

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Background CXC chemokine receptor 4 (CXCR4) is activated by phosphorylation (pCXCR4) and is essential for the migration of hematopoietic precursors to bone marrow. Data regarding the prognostic impact of CXCR4 in patients with B-acute lymphoblastic leukemia (B-ALL) are sparse and limited to the pediatric population. Patients and methods Fifty adult patients with B-ALL were included in the study. CXCR4 was assessed by flow cytometry and pCXCR4 by immunocytochemistry. All patients received a hyper-CVAD regimen. Results Thirty-one patients had positive CXCR4 expression, of whom only 18 (58%) had positive pCXCR4 expression. The complete response rate for patients with positive expression of pCXCR4 was significantly inferior to those with negative expression (61.1 vs. 93.7%; P < 0.01). In the multivariate analysis, pCXCR4 expression was associated with a worse disease-free survival ( P < 0.027) and overall survival (P < 0.024). Positive expression of pCXCR4 was not associated with an increased incidence of extramedullary disease or had any relation to the degree of maturity of B-ALL. Conclusion The current study indicates that adult B-ALL patients with positive expression of pCXCR4 have an inferior response to chemotherapy, disease-free survival, and overall survival compared with patients with negative expression.

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