Background Immune thrombocytopenic purpura (ITP) is a heterogeneous immune-mediated disorder in which the immune system reacts with cytokine-mediated T lymphocytes and a platelet autoantigen(s). Aim The aim of this study was to detect helper T-1 and T-2 response through assessment of tumor necrosis factor (TNF)-α and interleukin (IL)-6, respectively, and immunoglobulin (Ig)M, IgG, and IgA in patients with acute and chronic ITP and to detect their possible roles as predictors of the course of the disease. Patients and methods This cross-sectional case–control study, conducted at Ain Shams University, recruited 20 patients with ITP (10 acute and 10 chronic) and 10 age-matched and sex-matched healthy controls. All were subjected to detailed clinical assessment, assessment of serum IL-6 and TNF-α using enzyme-linked immunosorbent assay, and serum IgA, IgM, and IgG using nephlometric method. Results Mean serum values of IgM and IgG were statistically lower in patients with acute ITP, and statistically higher serum values of TNF-α were detected in patients with acute and chronic ITP compared with controls. IL-6 levels were statistically higher in acute ITP when compared with chronic ITP and with controls and a higher significant value in chronic ITP than controls. There were significant positive correlations between IgM, IgG, and platelet count and a significant negative correlation between both TNF-α and IL-6 and platelet count. On follow-up of patients with acute ITP, one of them developed a chronic progressive course, and this patient was found to have low serum levels of IgA and IgG at presentation. Conclusion Children with primary ITP had dysfunction at cytokine and serum Ig level.

Background/aim Chronic immune thrombocytopenic purpura (ITP) is an immune-mediated platelet destruction, with decreased platelet count to less than 100×10⁹/l for more than 12 months. Overall, 5–10% of children with chronic ITP experience serious bleeding, and their treatment is challenging. Rituximab is one of treatment options. Our aim is to evaluate low-dose rituximab in the management of childhood chronic ITP. Patients and methods Twenty-eight children with primary chronic ITP were prospectively administered rituximab at a dose of 100 mg/week for 4 weeks and followed up for 1 year. Results The initial response showed that 12/28 (42.8%) achieved complete remission, 2/28 (7%) minimal remission and 14/28 (50%) no remission. No significant differences were detected between responders and nonresponders regarding age and sex (P=0.94 and 0.59, respectively), except that the duration of the disease was significantly longer among nonresponders (P=0.025). The outcome after 1-year follow-up was as follows: one patient lost to follow-up, 14/28 (50%) were nonresponders, 8/28 (28%) relapsed, and 6/28 (21%) maintain sustained remission. Conclusion Rituximab is one of treatment choices for chronic ITP in children with convincing initial results; however, failure to achieve sustained remission is still a problem.

Background Hypercoagulability in chronic hemolytic anemia, namely, β-thalassemia major (TM), β-thalassemia intermedia (TI), and sickle cell disease (SCD), is well recognized. Activation of coagulation results in thrombin formation which in turn is inactivated by complex formation with its major inhibitor thrombin antithrombin complex (TAT). As a result, TAT is considered a coagulation marker which confirms the hypercoagulability state. Aim The aim of this study was to measure TAT as a coagulation activation marker in patients with β-TM, β-TI, and SCD and to correlate TAT levels with their clinicolaboratory parameters. Patients and methods A total of 60 children and adolescents having β-thalassemia syndromes and SCD were recruited from pediatric hematology clinic, Ain Shams University. They underwent routine complete blood picture and hemolytic profile in addition to TAT. Results The TAT was significantly higher in all patients (164.13±42.47 µg/l) compared with controls (106.75±21. 35 μg/l). In the thalasssemia group, the TAT level was 156.45±42.71 µg/l, whereas in patients with SCD, it was 179.50±38.52 µg/l. On comparing patients with thalassemia and those with SCD, there was a significant difference (P<0.05). However, measured TAT levels in patients with TM versus those with TI were 151.11±26.07l versus 167.54±65.25 µg/l, respectively. Comparison of TAT level between patients with TM and TI revealed no statistically significant difference. TAT was significantly correlated with mean corpuscular volume, mean corpuscular hemoglobin, and total and indirect bilirubin levels in all patients. Conclusion β-Thalassemia syndromes and SCD are associated with increased coagulation activity, and this activity is correlated with the level of hemolysis.

Background Rectal cancer is one of the most frequent human malignant neoplasms and the second most common cancer in the large intestine. Neoadjuvant concurrent chemoradiotherapy along with surgery has been strongly recommended for locally advanced cancers located in the middle or the distal rectum. Anemia is reported to have adverse effects on survival in cancer patients. The aim of this study was to evaluate the influence of anemia on radiochemotherapy treatment outcome in these patients. Patients and methods A total of 50 consecutive patients with histologically confirmed adenocarcinoma of the rectum were treated radically with three-dimensional conformal radiation therapy with concurrent 5-fluorouracil or 5-fluorouracil derivatives-based concurrent chemotherapy. The influence on the pathological response of hemoglobin concentrations at presentation before neoadjuvant concurrent chemoradiotherapy was studied. Results Out of 50 patients 22 were anemic at presentation. There was a statistically significant difference, P value of 0.001, in anemic and nonanemic groups for tumor downstaging, whereas nodal downstaging did not reach statistical significance (P=0.201). In the multivariate analysis, the circumferential resection margin was found to be the most important risk feature, with a local control rate of 95.7% in patients with a negative margin and a 25% local control rate in the patients with a positive margin, with a significant P value of 0.002. Conclusion Pretreatment anemia had negative effects on pathological response and local control among patients who underwent neoadjuvant chemoradiotherapy and surgery for rectal cancer. Strategies maintaining hemoglobin levels within the normal range could potentially be used to improve local control in rectal cancer patients.

Background Human neutrophil-specific antigen CD177 has an important role in various hematological, clinical, and immunological syndromes. Individuals with thalassemia were found to show an elevation in CD177 expression, which may be correlated with hematopoietic activity. Patients and methods Our case–control study was carried out at Clinical Pathology and Pediatric Hematology Departments, Zagazig University Hospitals, Egypt. This study was conducted on 80 (68) patients divided into two groups − group 1 included 34 patients diagnosed with β-thalassemia major, and group 2 included 34 apparently healthy individuals (control). All were subjected to routine clinical, laboratory, and radiological examination, as well as special laboratory examination: CD177% and mean fluorescence intensity (MFI) of gated neutrophils by flow cytometry and soluble transferrin receptor (sTFR) by enzyme-linked immunosorbent assay technique. Results There was a statistically significant difference as regards CD177 (MFI) and sTFR in thalassemia patients when compared with the control group (P<0.001). There was no significant correlation between CD177 (MFI) or percentage with both fetal hemoglobin and sTFR. Receiver operating characteristic curve analysis revealed that CD177 (MFI) is more sensitive but less specific than sTFR. Conclusion CD177 on gated neutrophils was increased in thalassemia patients but not correlated with their erythropoietic activity.

Background Primary immune thrombocytopenia (ITP) is a common hematological disorder of unknown etiology. DNA methylation is a major epigenetic modification of the DNA. It has a golden role in gene expression. It is mediated by DNA methyltransferases (DNMTs). The promoter of DNMT3B gene contains some single-nucleotide polymorphisms (SNPs) including that at position −149 (C/T), which was suggested to be implicated in the genetic susceptibility to ITP. The DNMT3A −448 G/A SNP in the gene promoter was found to have a protective effect against systemic lupus erythematosus. Aim The aim of the study was to investigate the association between DNMT3A −448 G/A SNP (rs1550117) and DNMT3B −149C/T SNP (rs2424913), and the risk for primary ITP and to evaluate the association between these SNPs and patients’ response to therapy. Participants and methods This prospective case–control study was conducted on 60 primary ITP patients and 30 healthy age-matched and sex-matched controls. Genotype analysis of DNMT3A −448 G/A and DNMT3B −149C/T was done using PCR-restriction fragment length polymorphism. Results The frequency of the DNMT3A −448 G/A SNP variant A-allele was significantly decreased in primary ITP patients compared with controls (odds ratio=0.829, 95%CI=0.097–0.964). DNMT3B −149C/T SNP variant T-allele was significantly higher in ITP patients with almost double-fold increase in the risk of ITP in comparison to controls (odds ratio=1.731, 95%CI=1.121–2.582). Conclusion The DNMT3A −448 SNP variant A-allele might has a protective effect against ITP. Also, the DNMT3B −149 SNP variant T-allele could be considered as a molecular risk factor for ITP.

Background The Philadelphia chromosome is the hallmark of chronic myeloid leukemia (CML). As the disease progresses to accelerated phase and blast crisis (BC), clonal evolution may occur with the emergence of additional chromosomal abnormalities. Wilms’ tumor 1 gene (WT1) plays an important role in leukemogenesis, and its expression could represent a useful molecular marker of hematological malignancies. Aim The aim of this study was to evaluate WT1 expression and compare its kinetics with that of BCR-ABL1 expression in peripheral blood of patients with CML, to explore the utility of WT1 as an alternative marker for prediction of early disease progression and sudden transformation to BC. Patients and Methods A total of 49 newly diagnosed patients with CML, and 20 normal individuals as controls were enrolled in this study. WT1 and BCR-ABL1 expression was evaluated by quantitative real-time PCR. Results There was significant correlation between the expression of WT1 and BCR-ABL1 only at diagnosis and after 3 and 6 months of therapy. At 12 months of follow-up, an increase in %WT1 associated with low BCR-ABL1 was noticed in two of cases. One case suddenly progressed to BC at 18 months, and the other case showed emergence of clonal chromosome abnormality (+8) and progressed to accelerated phase. Conclusion Our results suggested that serial assessment of WT1 transcript level in patients with CML may be a useful marker for predicting early and sudden disease progression into advanced stages.