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  Citation statistics : Table of Contents
   2016| October-December  | Volume 41 | Issue 4  
    Online since January 20, 2017

 
 
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ORIGINAL ARTICLES
Joint health in Egyptian children with hemophilia A: what are the affecting factors?
Atwa T.H. Zeze, Eldash H Hanaa, Telep R Nejm Eldin
October-December 2016, 41(4):168-173
DOI:10.4103/1110-1067.198651  
Background Hemophilia A is an X-linked recessive disorder. Patients experience spontaneous and trauma-induced bleeds. Recurrent joint bleeds lead to progressive disability. Attaining healthy joint is the main goal of hemophilia treatment. The objective of our study was to evaluate joint health and factors affecting it in hemophilia patients who were on episodic treatment. Methodology This is a prospective observational study. Hemophilia joint health score was performed on 42 children with severe hemophilia who were selected from pediatric hematology clinics. Factors that may affect hemophilia joint health score were evaluated. These factors were age, age at first joint bleed, frequency of hemarthrosis per year, BMI, parents’ level of education, residence, hepatitis C virus infection, performing physiotherapy regularly, and performing conservative measures upon active bleeding (ice, rest, compression, and elevation). Data were analyzed using Statistical Package for Social Sciences. Results The mean total joint score was 12.3±7.3. Knee, ankle, and elbow were the target joints in 28.6, 26.2, and 26.2% of patients, respectively. Older patient age, earlier age at first hemarthrosis, and frequent hemarthrosis were associated with higher joint score. Performing physiotherapy was associated with a lower joint score. No significant effects could be elicited as regards other studied factors. Conclusion Older patients and those with frequent hemarthrosis were more prone to joint damage. Performing physiotherapy should be encouraged, as it can improve joint health in hemophilia patients.
  1 1,267 244
Leukoreduced red blood cell: storage-related complement regulatory proteins and CD47
Manal H Farahat, Mohammad A Sharaf
October-December 2016, 41(4):200-205
DOI:10.4103/1110-1067.198654  
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.
  1 833 101
Dipeptidyl peptidase-4 (CD26): a prognostic marker in patients with B-cell chronic lymphocytic leukemia
Hossam Hodeib, Abeer Shahbah
October-December 2016, 41(4):206-210
DOI:10.4103/1110-1067.198650  
Introduction B-cell chronic lymphocytic leukemia (B-CLL) is an incurable disease; nevertheless, its prognosis varies widely among patients. CD26 is considered a cell adhesion molecule. It is suggested that CD26 expression is involved in tumor growth, invasion, and metastasis. Aim The aim of this study was to assess the prognostic value of dipeptidyl peptidase-4 (CD26) in patients with B-CLL and its relation to the clinical and laboratory parameters. Patients and methods This case–control study was carried out from April 2013 to April 2016, and it was performed on 75 newly diagnosed B-CLL patients admitted at Hematology and Oncology Unit, Department of Internal Medicine, Tanta University Hospital, Egypt; 30 apparently healthy individuals were involved in the study as the control group. Dipeptidyl peptidase-4 (CD26) expression was evaluated by flow cytometry. Results The main finding in this study was that CD26 expression was increased in B-CLL patients in comparison with normal subjects. There was also a positive correlation between white blood cell count, absolute lymphocytosis, clinical staging of B-CLL patients, and CD26 expression. Overall survival (OS) and disease-free survival (DFS) were shorter in B-CLL patients with positive CD26 expression compared with patients with negative CD26 expression. Patients with positive CD26 expression had significantly shorter OS (16 vs. 20 months) and significantly lower DFS (6 vs. 16.5 months) at 24 months compared with those with negative CD26 expression. Conclusion CD26 expression was significantly higher in B-CLL patients compared with healthy subjects, and its expression among B-CLL patients correlated with poor laboratory data, clinical staging, OS, and DFS, and thus it may play a role in prognosis of B-CLL. CD26 could be used as a prognostic marker either alone or in combination with other markers in assessment of B-CLL patients.
  1 778 142
Novel immunophenotyping marker in acute myeloid leukemia: does it implicate prognosis?
Amira Y Abd El-Naby, Amr Gawaly, Shereen Awni
October-December 2016, 41(4):155-160
DOI:10.4103/1110-1067.198646  
Introduction AML is a heterogeneous group of neoplasms that affect hematopoietic cells responsible for the production of myeloid lineages in bone marrow (BM). Novel immunophenotyping marker in AML (CD30) is a 120 kDa cell membrane glycoprotein that shares sequence homology with tumor necrosis factor (TNF) receptors; it is expressed by myeloblasts (CD34+ and/or CD117+) in a substantial number of cases of non Monocytic AML, either de novo or arising from MDS. CD30 expression is more common in AML patients with unfavorable chromosomal abnormality. It is suggested that CD30 could be a target for therapy by using anti-CD30 antibodies in a subset of patients with non Monocytic AML; also it might provide useful information for patient prognosis and stage of disease. Aim of work Is to assess novel immunophenotyping marker in AML (CD30) and cytogenetic abnormality and its correlation with prognosis. Materials and methods This prospective cohort study included 120 patients with newly diagnosed acute myeloid leukemia. Their ages ranged from (2 to 75) years with a mean of (40.15±23.55) years. Chromosomal analysis by karyotyping was done in all AML patients and Multicolor flow-cytometry immunophenotypic analysis was performed on bone marrow aspirates of the AML patients using fluroisothiocyanate (FITC) conjugated antibodies to assess CD30 expression on BM myeloblasts. Results The previous study showed that CD30 was expressed in all AML cases rather than M4 and M5 cases that showed negative expression. In addition, this study showed that there is more CD30 expression in myeloblasts with unfavorable chromosomal abnormalities. A significant association between platelets counts and CD30 expression was also observed. There was a higher degree of thrombocytopenia and a greater tendency to have higher leucocytic counts in patients having +ve CD30 expression than those with –ve CD30 expression. Conclusion The analysis of CD30 expression has a potential role to be used as a prognostic marker in AML.
  - 1,367 3,169
Value of human CLEC12A expression in acute myeloid leukemia
Gehan M Hamed, Mona F Abdel Fattah
October-December 2016, 41(4):161-167
DOI:10.4103/1110-1067.198648  
Background The need for identification of new specific, stable antigens during the course of acute myeloid leukemia (AML) is warranted to improve diagnosis, relapse detection, and eradication of leukemic cells. Aim We measured the surface marker human C-type lectin domain family 12, member A (CLEC12A), in 60 AML patients, 24 acute lymphoblastic leukemia (ALL) patients, and 20 controls by flow cytometry, to determine its diagnostic utility and stability in AML. Results CLEC12A was positively expressed in all studied AML patients, negative expression was detected in ALL patients, and normal CD34+ cells of the controls with significantly higher mean % expression and median fluorescence intensity was detected among AML patients (P<0.001). Receiver operating characteristic curve analysis revealed that CLEC12A at a cutoff value of at least 15.1% can diagnose and differentiate between AML and ALL with 100% sensitivity and specificity. CLEC12A was found to be positively expressed both in children and adult AML patients with significantly higher mean % expression and median fluorescence intensity in children (P=0.046), and a significant difference was found between different French–American–British Classification subtypes (P<0.001). No significant difference was detected as regards sex, newly diagnosed untreated AML patients versus AML patients in relapse (79.1±11.7 vs. 67.4±19.3, P=0.052), CD34+ versus CD34− leukemic blast cells (75.1±20.7 vs. 74.1±21.7; P=0.899), or between different cytogenetic prognostic risk groups (P>0.05). The marker was found to be positively expressed without significant difference in paired diagnosis/relapse samples, indicating its stability during the course of disease and after treatment. Conclusion CLEC12A is a specific and stable diagnostic marker of AML that could improve leukemia-associated immunophenotypes both in CD34+ and the poorly characterized CD34− patients by flow cytometry. In addition, low expression of CLEC12A on normal CD34+ progenitor cells positions the marker as a potential therapeutic target.
  - 1,190 1,360
Hematological and biochemical parameter alteration after plateletpheresis donation
Manal H Farahat, Mohammad A Sharaf
October-December 2016, 41(4):174-179
DOI:10.4103/1110-1067.198652  
Background The plateletpheresis process is a great improvement in transfusion medicine, and it is thought to be generally safe to the donor. However, donors’ safety issues and the anticoagulant used during these procedures have not been fully explored. This study aimed at analyzing the significance of alteration in some hematological parameters, total calcium and total magnesium levels, in donors after plateletpheresis procedure. Materials and methods The plateletpheresis procedures were performed on 72 donors. Prehematological and posthematological values were analyzed, such as hemoglobin concentration, hematocrit, platelet, white blood cell, red blood cell, mean platelet volume, and platelet distribution width, as well as measured total serum calcium (tCa2+) and magnesium levels (tMg2+), at different time intervals during and after the procedure in all donors. Results After plateletpheresis procedures, we observed that the hemoglobin concentration, hematocrit%, and white blood cell count were highly significantly decreased (P<0.000), platelet count was decreased significantly (P<0.001) in the donors, and there was a significant slight increase in mean platelet volume (P=0.036), without significant changes in platelet distribution width and red blood cell. In addition, there were significant decrease (P<0.05) in mean total serum calcium and magnesium levels after 60 min from the procedure onset compared with values of baseline levels, and their levels increase again near baseline levels 30 min after the procedure. Discussion Plateletpheresis procedures are very safe for donors; however, indicated biochemical and hematological parameters before and after the donation are definitely useful to establish postdonation reference ranges that are needed for carefully monitoring, retaining and following-up the donor, reviewing the repeated donations, and to protect donation safety by looking for the cumulative effects of these changes, as well as transfusion of blood products with higher-quality needs.
  - 1,205 179
Apolipoprotein M and transforming growth factor-β levels as predictive biomarkers in idiopathic recurrent venous thromboembolism
Hossam Hodeib, Ola Elshora, Abeer Shahbah, Eman Ramadan, Ahmed Esam, Mohamed Hantera, Ahmed A Elshoura
October-December 2016, 41(4):180-186
DOI:10.4103/1110-1067.198653  
Introduction Venous thromboembolism (VTE), including deep vein thrombosis (DVT) and/or pulmonary embolism (PE), is a common, acute, multifactorial, fatal disease, although treatable. The recurrence rate of VTE is about 17% after 2 years of follow-up and 30% after 8 years. Apolipoprotein M (ApoM) and transforming growth factor-β (TGF-β) could be prognostic biomarkers of recurrence of VTE. Aim of the study The aim of this study was to assess the prognostic value of ApoM and TGF-β levels as predictors of recurrence of VTE among patients with first thrombotic event. Patients and methods This prospective cohort study was carried out from December 2013 to December 2015 in the Internal Medicine, Chest, Anesthesia, Surgical ICU, and Surgery Departments, Tanta University Hospital, Egypt, on 78 patients of both sexes and age younger older than 18 years who were admitted in the hospital for DVT or PE. DVT was confirmed with imaging techniques, venography or compression ultrasonography, and PE was confirmed with ventilation/perfusion lung scan. They were initially treated with low molecular weight heparin and then with warfarin as an oral anticoagulant for 3–6 months. They were observed for 2 years at 3-month intervals in the first year and every 6 months in the second year or until the time of recurrence of VTE. We evaluated the plasma levels of ApoM, TGF-β1, TGF-β2, and TGF-β3 at 3–12 weeks after withdrawal of anticoagulant therapy. Results The main finding in the present study was that the mean plasma levels of both TGF-β1 and TGF-β2 were significantly lower in patients with recurrence of VTE when compared with those without recurrence of VTE. However, the mean plasma levels of ApoM and TGF-β3 were not significantly different between patients with recurrence of VTE and those without recurrence of VTE. Conclusion TGF-β1 and TGF-β2 could be useful prognostic biomarkers in VTE patients and can predict the recurrence of the thrombotic event.
  - 709 81
Significance of neuropilin-1 mRNA expression in chronic myeloid leukemia
Hany A Labib, Rasha M Hagag, Sheren Elshorbagy, Ahmed A Alnagar, Neveen G Elantonuy
October-December 2016, 41(4):187-193
DOI:10.4103/1110-1067.198647  
Background Neuropilins (NRPs) are transmembrane glycoproteins that act as receptors for vascular endothelial growth factors and are involved in the process of tumor angiogenesis. Patients and methods We analyzed the significance of NRP-1 RNA expression level in 63 newly diagnosed chronic myeloid leukemia (CML) patients and 40 healthy controls using real-time PCR. Results NRP-1 was significantly highly expressed in CML patients than in controls and in patients in the accelerated phase than in those in the chronic phase. Its levels were significantly positively correlated with total leucocytic count (TLC), platelets count, and percentage of blast, whereas it was negatively correlated with progression-free survival. NRP-1 expression level revealed a statistically significant difference as regards response to imatinib therapy: it was significantly higher in those who did not achieve complete molecular response. During the follow-up period, the NRP-1 levels in patients still in remission were significantly lower than those who showed progression to accelerated or blastic phase; the median time of progression-free survival in patients with high NRP-1 was significantly shorter than those who had normal level. Conclusion We conclude that NRP-1 expression is significantly associated with CML and that its level might serve as an indicator for disease severity and progression.
  - 694 73
Serum-soluble APRIL influences survival in patients with chronic lymphocytic leukemia
Ola A El Shora, Abeer Shahbah
October-December 2016, 41(4):194-199
DOI:10.4103/1110-1067.198649  
Background A proliferation-inducing ligand (APRIL) is a member of the tumor necrosis factor family. APRIL plays an important role in B-cell development; it activates chronic lymphocytic leukemia (CLL) cells by reacting with its receptors, enhancing immune recognition, proliferation, and survival of the leukemia cells. Aim The aim of this study was to determine the impact of serum-soluble APRIL levels detected at diagnosis in B-CLL patients, and their relationship with disease parameters and patient outcomes. Patients and methods Serum APRIL levels were estimated in 40 untreated B-CLL patients and 40 apparently healthy controls. Serum was withdrawn at diagnosis of untreated CLL disease and was tested by enzyme-linked immunosorbent assay. Results APRIL levels in peripheral blood samples were significantly higher in B-CLL patients than in normal participants. There were positive correlations between serum-soluble APRIL level, age, white blood cell count, peripheral blood lymphocyte count, CD38%, ZAP-70, and modified RAI staging. There were negative correlations between serum-soluble APRIL, hemoglobin level, and platelet count. During a 2-year follow-up, APRIL levels correlated with overall survival and disease-free survival. Conclusion APRIL is a powerful prognostic marker related to parameters of disease activity and staging and, more importantly, to overall survival and disease-free survival.
  - 709 73