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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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Background Conventional diagnosis of hereditary red blood cell (RBC) membrane disorders, in particular hereditary spherocytosis (HS), is labor intensive, time consuming and requires at least 2 ml of blood, which might be impractical in the neonatal period. Participants and methods We evaluated the use of eosin-5-maleimide (EMA) as a rapid screening test for patients with HS. RBCs from 74 healthy controls and 66 anemic children (35 HS and 31 other hemolytic anemias; 10 cases diagnosed as thalassemia, eight cases of autoimmune hemolytic anemia, one case of ovalocytosis and 12 cases of undiagnosed hemolytic anemia) were stained with EMA and analyzed for their mean fluorescence intensity using flow cytometry. Results RBCs from patients with HS showed a greater degree of reduction in mean fluorescence intensity of EMA compared with those from normal controls and patients with other hemolytic diseases. These findings showed that the fluorescence flow cytometric-based method is a simple, sensitive and reliable diagnostic test for RBC membrane disorders using a small volume of blood, and results could be obtained within 2 h. Such a method could serve as a first-line screening for the diagnosis of HS in routine hematology.

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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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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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Background Isochromosome 17q (i17q) is a well-known nonrandom secondary anomaly in chronic myeloid leukemia (CML), which occurs either solely or with other additional anomalies. Objectives The aim of the study was to explore the influence of i17q in CML patients in different phases of the disease and the prognostic impact of acquiring such an anomaly on disease progression, outcome, and response to therapy. Materials and methods Cytogenetic analysis was carried out on 100 CML patients by G-banding and fluorescence in-situ hybridization using LSI BCR/ABL, LSI p53(17p13)/MPO (17q22) i(17q), CEP 8, and CEP Y probes. Results Isochromosome 17q was detected in 16% of cases. All examined bone marrow smears of i(17q)-positive patients were hypercellular and showed variable degrees of dysplastic changes mainly in myeloid lineage, in the form of hyposegmentation and hypogranulation, together with dysplastic features of megakaryocytes in 70% of them. A highly significant association of i(17q) with poor prognosis was confirmed statistically (P = 0.002) compared with the prognosis in negative patients. The event-free survival of the i(17q)-positive group was 1.6 months compared with 11.5 months in negative patients. However, no statistically significant association was revealed with standard prognostic factors (P>0.05). Conclusion Isochromosome 17q identifies a subgroup of CML with distinct clinicopathologic features and with high risk for aggressive disease progression.

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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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A case of plasmablastic lymphoma that has not been described previously in the thyroid is presented with its clinicopathological features and the diagnostic difficulties encountered. A detailed histopathology in conjunction with immunohistochemistry is recommended for appropriate diagnosis and management in the setting of HIV-negative status. Dealing with a case presenting primarily as a thyroid mass can be challenging in the scenario of a CD20-negative immunoprofile.

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Context Abnormalities in the control of apoptosis play an important role in tumorigenesis. Survivin is one of eight members of the inhibitor of apoptosis protein family that regulates and integrates cell division and suppresses apoptosis. Aim The aim of this study was to assess intracellular expression of survivin on malignant myeloid blast cells and its correlation with clinical outcome, overall survival (OS), and other prognostic factors among adult Egyptian patients with acute myeloid leukemia (AML). Settings and design A total of 120 patients with de-novo AML were treated and followed up in Ain Shams University Hospitals Hematology Units and compared with 60 age-matched and sex-matched normal healthy controls. Patients and methods All patients received induction chemotherapy under a 3 + 7 regime, whereas AML-M3 patients received an all-transretinoic acid-based regime. Detection of intracellular survivin antigen in myeloid blast cells was done by flow cytometry on bone marrow samples at diagnosis and after chemotherapy. Results Survivin expression was higher in AML patients at day 0 compared with healthy controls (P = 0.001). The highest survivin level was seen in AML French American British subtypes M5 and M4. A significant positive correlation was found between patients' age, CD15, CD14, and CD11c expression, whereas negative correlation was found between survivin level and the control group, which included 60 age-matched and sex-matched normal healthy volunteers under complete remission, and event-free survival. Patients with a positive survivin expression have shorter OS compared with patients with a negative survivin expression (log-rank = 3.940, P = 0.047). Conclusion Higher survivin levels at diagnosis predict poor response to chemotherapy and shorter OS (P = 0.016).

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Background Higher levels of growth hormone (GH) and insulin-like growth factor-1 (IGF-1) stimulate the growth of myeloid and lymphoid cells and may contribute to leukemogenesis. Aim The aim of this study was to evaluate serum GH and IGF-1 levels in children with acute lymphoblastic leukemia (ALL) during active disease and after achieving clinical remission. Patients and methods Forty children with newly diagnosed ALL on the basis of history, physical examination, and blood and bone marrow analysis were included in the study. Patients were treated with vincristine, prednisolone, intrathecal methotrexate, l-asparaginase, and adriamycin and followed up for 6 weeks, in order to achieve clinical remission. Twenty healthy children of the same age group were used as the control group. Serum GH and IGF-1 levels of both controls and patients before and after therapy were measured. Results After the diagnosis of ALL and before therapy, GH was found to be nonsignificantly higher compared with healthy controls (8.80 ± 6.80 vs. 5.76 ± 2.77 ng/ml, P = 0.061), but IGF-1 was significantly higher in patients than in controls (233.50 ± 100.1 vs. 179.70 ± 77.94 ng/ml, P = 0.040). After achieving remission with 6 weeks of therapy, GH was found to be highly significantly decreased (2.04 ± 1.31 ng/ml) in patients compared with the level before treatment and compared with the control group (P = 0.0001). Also, IGF-1 was reduced significantly (192.93 ± 81.15 ng/ml) compared with the level before treatment (P = 0.0001) but was nonsignificantly higher compared with the level of the control group (P = 0.569). There was significant correlation between GH and IGF-1 levels in ALL patients before therapy and after achieving remission (P < 0.05). Conclusion The higher levels of GH and IGF-1 during active ALL and their reduction after treatment may be helpful in assessing the disease activity and predicting the response to chemotherapy.

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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 Haemophilia-A (HA) is an X-linked recessive disorder characterized by the deficiency of functional clotting factor VIII resulting in lifelong bleeding diathesis. We predict that HA would be associated with iron deficiency and the risk will be higher in those with severe disease. If our prediction is correct, the frequency and the relative risk (RR) of iron deficiency will be higher in patients with severe HA in comparison with nonsevere HA. Materials and methods We evaluated the levels of haemoglobin concentrations, red cell indices and serum ferritin retrospectively with respect to the disease severity among a cohort of treatment-naive patients with HA as seen at the time of diagnosis in some hospitals in northern Nigeria. Results Out of the 39 patients studied, 19 were iron deficient, yielding an overall frequency of iron deficiency of 48.7%. Out of the 39 patients, 24 (61.5%) had severe HA and 15 (38.5%) had nonsevere HA. Patients with severe HA had a significantly higher frequency of iron deficiency in comparison with nonsevere HA (66.7 vs. 20%, P < 0.004) and the RR of iron deficiency for patients with severe HA was 2.6 (95% confidence interval: 1.9-3.4, P = 0.003). Conclusion Iron deficiency is very common among patients with HA, the frequency and the RR of which is higher among patients with severe disease. Therefore, patients with HA should be regularly screened and treated for iron deficiency to prevent the adverse impact of iron deficiency on wound healing, the immunity and the mental development of haemophilic patients. However, the possibility of undesirable side effects of iron such as erosive gastritis, which can increase the risk of gastrointestinal haemorrhage in HA patients, calls for caution in the choice of oral pharmaceutical iron preparations vis-à-vis nonpharmaceutical dietary supplementation. Healthcare centres should formulate standard guidelines for the effective and safe treatment of iron deficiency in haemophilia patients.

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Introduction Chronic lymphocytic leukemia (CLL) is the most common type of adult leukemia in the western world. It is a malignancy of mature B cells involving the blood, bone marrow (BM), and lymphoid tissues, and its cells arise from polyclonal expansion of CD5+ B lymphocytes transformed into a monoclonal population by mutational agents. CD69 is an integral membrane protein belonging to the lectin family. It is expressed after activation in all BM-derived cells except erythrocytes. CLL exhibits features of activated and antigen-experienced B lymphocytes and CD69 overexpression. CD69 is significantly correlated with poor clinical and biological prognostic factors, and this supports its introduction into routine laboratory assessment and, possibly, in a prognostic scoring system for CLL after an adequate standardization process. Objective The aim of this study was to detect CD69 expression in newly diagnosed patients with CLL by flow cytometry and correlate it with clinical and laboratory parameters to evaluate it as a prognostic factor. Patients and methods This study was conducted on 40 B-CLL patients who attended Ain Shams University Hospitals over 1 year. All patients were subjected to full medical history and clinical examination, RAI staging according to disease burden and the degree of BM involvement, abdominal ultrasonography, complete blood count with examination of peripheral blood smears, BM aspiration with morphological examination, and immunophenotyping of BM or whole peripheral blood applying monoclonal antibodies CD20, CD79b, FMC7, serum IgM, CD5/CD19, CD10, CD103, CD123, CD23, and CD38, and κ and λ light chains and CD69 expression. Results In the current study all of the studied B-CLL patients expressed CD69. Among the 40 studied patients 23 had high CD69 expression (group I) and 17 had low CD69 expression (group II). A highly significant elevation in group I patients compared with group II patients was found (P = 0.000). A highly significant reduction in hemoglobin (Hb) level (P = 0.001), elevation in lactate dehydrogenase concentration (P = 0.006), elevation in RAI staging, and elevation in CD38 expression (P = 0.005) were observed in group I. A highly significant negative correlation was found between CD69% and Hb level (P = 0.008) and platelet count (P = 0.009). A highly significant positive correlation was found between CD69% and hepatomegaly (P = 0.008) and RAI stage (P = 0.008) and significant positive correlation was found between CD69% and CD38% expression (P = 0.012). An association study was conducted between CD69% expression and all the standard prognostic factors in B-CLL patients. There was a highly significant association between CD69% expression and presence of hepatomegaly (P = 0.002) and a significant association between CD69% expression and presence of splenomegaly (P = 0.028) and low Hb levels (P = 0.013). Conclusion Several clinical and biological variables have been reported to predict the outcome of CLL patients, such as advanced patient age, male sex, higher absolute lymphocyte count, greater extent of lymphadenopathy, and raised serum β2-microglobulin levels, all of which are associated with inferior prognosis. In the current study all of the studied B-CLL patients expressed CD69. CLL patients were divided into two subsets with significantly different prognosis: those with high CD69 (group I) (≥30%) and those with low CD69 (group II) (<30%) expression; both were compared with respect to the different studied parameters. There was highly significant elevation of CD69 in group I compared with group II. There was a highly significant reduction in Hb level and elevation in lactate dehydrogenase concentration in group I patients in comparison with group II patients. A highly significant relation was found between the two groups with respect to RAI staging: an advanced RAI stage was found among group I patients in comparison with group II patients. The current study evaluated CD69 as a prognosticator and studied the significant association of its high expression with the standard prognostic factors, notably splenomegaly (P = 0.028) and hepatomegaly (P = 0.002), low Hb level, low platelet count, and advanced RAI stage. CD69 expression is associated with both immunoglobulin variable heavy chain mutation status and survival. It is recommended to perform a larger prospective study on CD69 expression in B-CLL patients, studying its relation to overall survival and progression-free survival and its re-evaluation during the course of treatment as we recommend assessment of this marker by flow cytometry among independent prognostic markers in B-CLL.

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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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Background There are plenty of studies on prevalence of anaemia in high-risk groups such as pregnant and lactating women and children. Reliable data on the prevalence and causes of anaemia in the elderly population are not available, particularly in this region. Thus, the present study was carried out to profile anaemia in geriatric patients in our setup. Objectives The aim of this study was to profile anaemia clinicohaematologically among elderly. Materials and methods A total of 168 geriatric patients aged 65 years and above, male and female patients with haemoglobin less than 12 g/dl admitted in the Tertiary Care Hospital, were included in the study. Investigations were carried out, mainly haematological with supporting biochemical parameters, which included complete blood count, peripheral blood film examination, serum B12 and folate studies, bone marrow cytology, iron studies, liver and renal function tests, urine examination and radiological examination when required. Detailed clinical history, systemic examination and complete haematological, biochemical and radiological investigations were carried out when patients were being managed in the respective wards/units. Results Various underlying pathologies encountered were nutritional deficiency anaemia (47.6%), anaemia of chronic disease (20.2%), bone marrow infiltration (8.3%), multiple myeloma (7.1%), myelodysplastic syndrome (4.8%), myelofibrosis (4.8%), acute myeloid leukaemia (3.6%), anaemia of renal disease (2.4%) and chronic lymphocytic leukaemia (1.2%). Anaemia associated with chronic diseases included cases of pulmonary tuberculosis, rheumatoid arthritis, bronchiectasis, pneumonia and ischaemic heart diseases, which predisposed the patient to greater morbidity. Conclusion The incidence of anaemia is quite high among elderly patients, more so when associated with chronic diseases and malignancies. The major cause found is nutritional anaemia due to deficiency of iron, folic acid/vitamin B12 or dual deficiency. It is very important to diagnose the cause of anaemia by detailed investigations before initiating the required therapy. Egypt J Haematol 39:-0 © 2014 The Egyptian Society of Haematology.

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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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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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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 Pregnant women are more prone to oxidative stress. Iron deficiency anemia not only affects hematological parameters but also disturbs body oxidative balance, which impairs pregnancy outcome. Besides, iron therapy may generate harmful oxygen species. Objectives The aim of this study was to investigate the effect of the nature of oral iron supplementation (ferrous vs. ferric) in pregnant women with iron deficiency on oxidative stress and its correlation with peripheral systemic inflammatory response markers. Patients and methods A clinical trail study involves study included 30 healthy and 50 anemic pregnant women in their 20th-36th gestational weeks who fulfilled the inclusion criteria. They were randomly distributed to receive either ferrous sulfate or ferric polymaltose complex. Outcome was assessed after 8 weeks of iron supplementation and included hematological parameters, neutrophil : lymphocyte ratio, platelet : lymphocyte ratio, mean platelet volume, serum malondialdehyde (MDA), total antioxidant capacity (TAC), iron, and ferritin. Results Anemic pregnant women have increased oxidative stress with high levels of MDA and TAC, both at baseline and following iron supplementation (P < 0.001). Following 8 weeks of iron supplementation, there were a significant increase (P < 0.001) in hemoglobin and serum ferritin, and a significant decrease (P < 0.001) in the peripheral inflammatory markers neutrophil: lymphocyte ratio, platelet: lymphocyte ratio, mean platelet volume, and absolute lymphocyte counts. Conclusion Iron polymaltose complex was as effective as ferrous sulfate as both were able to correct hematologic parameters in parallel to a decrease in MDA and peripheral inflammatory markers together with an increase in TAC levels to maintain oxidative balance.

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Background Little is known about the prognostic importance of coexpression of CD49d and CD38 in chronic lymphocytic leukemia (CLL) patients. Aim This study aimed to investigate the coexpression of both CD38 and CD49d as prognostic and survival markers in CLL patients. Patients and methods Fifty-two patients with newly diagnosed B-cell CLL were included in the study. Twenty age-matched and sex-matched healthy control participants were also included in the study. Patients were subjected to a clinical examination and abdominal ultrasonography and chest radiography. Laboratory investigations including complete blood count, β2 microglobulin, cytogenetic analysis, and immunophenotyping by flow cytometer (B-lymphocyte markers and the expression of CD38 and CD49d) were performed. Results There was a significant decrease in hemoglobin concentration and platelet counts in patients who coexpressed CD49d+/CD38+ compared with patients who expressed CD49+ alone, whereas white blood cell and lymphocyte counts, lactate dehydrogenase, and β2 microglobulin were significantly higher. In addition, CD49d+/CD38+ coexpression was significantly high in advanced stages of CLL. A positive correlation was detected between CD49d expression and poor prognostic parameters in CLL. The median treatment-free time was shorter in CD49d+ patients (32 months) compared with CD49d- patients (98 months). The median treatment-free duration was shorter in CD38+ patients (28 months) compared with CD38- patients (102 months). In the concordant cases of CD49d+/CD38+, the median treatment-free survival was shorter (24 months) in patients with CD49+/CD38+ patients compared with disconcordant cases of CD49d+/CD38- patients (62 months). Conclusion CD38 and Cd49d expressions are considered prognostic markers for CLL patients and they should be assessed to decided on the patient's therapy and to determine disease prognosis. These molecules should also be tested in a large-scale study to determine their potential in preventing frequent relapses and development of resistance to chemotherapy in CLL.

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Background Despite progress in red blood cell (RBC) storage, storage lesions still occur, which lead to complement activation and removal of RBCs from circulation. The aim of this study was to assess the surface concentration of complement regulatory proteins, as well as CD47, on leukoreduced RBCs collected on the atreus device and stored in sodium–adenine–glucose–mannitol medium for 42 days. Materials and methods We studied the surface expression of leukoreduced RBC C3d, CD35, CD55, CD59, and CD47 using flow cytometry on weekly intervals from day 1 to day 42 of storage. Results We observed a nonsignificant increase of surface C3d levels (P=0.146) and a decrease in CD47 overall storage time (P=0.196). There was a significant decrease demonstrated in CD35 (P=0.025), and a strong significant decrease in CD55 in their expression over storage time (P=0.000). Last, a nonsignificant alteration of CD59 was observed (P=0.353). Conclusion The study of atreus-processed leukoreduced RBC units stored in sodium–adenine–glucose–mannitol medium for surface expression of complement regulatory proteins showed its inhibition until toward the end of storage time, and also showed a good preservation of CD47, which was thought to be important in transfused RBC survival. However, more research is potentially needed in areas of complement evaluation during component processing.

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Background A wide array of ethnomedicinal values have been attributed to the different parts of Annona muricata, and indigenous communities in Nigeria, Africa, and South America extensively use this plant to augment conventional drugs. Aim The beneficial effects of A. muricata on the hematological profile have also been widely reported and this research sought to validate these claims. Design Adult albino Wistar rats were used in this study. Methanolic extracts of the various parts of the plant were used, with which we determined the 50% lethal dose (LD₅₀) and acute toxicity status of the plant before hematological studies for a duration of 28 days (subchronic studies). Materials and methods During the subchronic studies, 100, 200, 400, 600, and 800 mg/kg of the fruit, leaf, stem-bark, and root-bark methanolic extracts were administered to groups 2–6, respectively, whereas group received 2 ml of distilled water and served as control. At the end of the administration period, the rats were killed and blood samples collected for onward hematological studies. Phytochemicals were quantified using standard procedures. Results The obtained results showed that both the leaf and fruit extracts had LD₅₀ of 1918.33 mg/kg, whereas the stem-bark and root-bark both had LD₅₀ above 5000 mg/kg. Subchronic observations were also made, including increased heart rates and diarrhea. The fruit and stem-bark extracts recorded a dose-dependent increase in CD4⁺ cells, especially from the 200 mg/kg dose. Also, the fruit, leaf, and root-bark extracts showed a dose-dependent increase in white blood cells and lymphocytes. The extracts of the various parts of the plant apart from the stem-bark recorded marked increase in platelet levels. The various extracts of the plant parts recorded striking increase in red blood cells, hemoglobin concentration, and packed cell volume. These observations could be linked to the remarkable quantity of alkaloids, flavonoids, and phenols present in the leaf and fruit fractions. Conclusion Thus, the obtained data suggest and validate the reported hemomodulatory and wound-healing properties of A. muricata, especially the fruit and leaf.

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Background β-thalassemia major (β-TM) is a common inherited hemolytic type of anemia. Repeated blood transfusions predispose β-TM patients toward peroxidative tissue injury because of secondary iron overload. Objectives This study aimed to evaluate the effects of iron overload on antioxidant enzymes and liver cell damage in β-TM patients undergoing regular blood transfusions. Patients and methods This prospective case–control cohort study included 30 pediatric patients with a confirmed diagnosis of β-TM on regular blood transfusions and 20 age-matched and sex-matched healthy children attending the Qena University Hospital, Pediatric Clinic. Blood samples were withdrawn from each patient to measure serum levels of ferritin, glutathione peroxidase (GPX), and superoxide dismutase (SOD). Results Total bilirubin, aspartate transaminase (AST), alanine transaminase (ALT), and ferritin levels were significantly higher in the β-TM group (P<0.001, 0.001, 0.001, and 0.001, respectively), whereas GPX and SOD were significantly lower in the β-TM group (P<0.001 and 0.001). The correlation between serum ferritin level and age, bilirubin, AST, and ALT in patients group showed that, the correlation between serum ferritin level and age was (0.745) while P-value was <0.001, the correlation between serum ferritin level and bilirubin level was (0.665) while P-value was <0.001, the correlation between serum ferritin level and (AST) level was (0.727) while P-value was <0.001 and the correlation between serum ferritin level and (ALT) level was (0.737) while P-value was <0.001. The correlation between SOD and age, ferritin, bilirubin, AST, and ALT in patients group in patients group showed that, the correlation between (SOD) and age was (−0.454) while P-value was 0.012, the correlation between (SOD) and ferritin level was (−0.664) while P-value was <0.001, the correlation between (SOD) and bilirubin level was (−0.535) while P-value was 0.002, the correlation between (SOD) and (AST) level was (−0.567) while P-value was <0.001 and the correlation between (SOD) and (ALT) level was (−0.558) while P-value was <0.001. Conclusion Impaired levels of antioxidant enzymes SOD and GPX in patients with β-TM on repeated transfusion, in addition to excessive free iron concentration, iron overload may attribute to oxidative damage in these patients. Antioxidant systems that compensate for reduced lipid peroxidation to lower tissue damage are needed.

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