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   2015| April-June  | Volume 40 | Issue 2  
    Online since July 22, 2015

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The frequency of iron deficiency among patients with haemophilia-A in northern Nigeria: correlation with the disease severity and clinical implications
Sagir G Ahmed, Modu B Kagu, Umma A Ibrahim, Audu A Bukar
April-June 2015, 40(2):85-89
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.
  2,386 209 2
Iron status and erythropoiesis in chronic hepatitis C patients on hemodialysis
Ashraf M El Hefni, Reda A Kamel Salem, Huda Ebian
April-June 2015, 40(2):80-84
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.
  1,499 192 1
Frequency of the ALK gene and its prognostic value in neuroblastoma by FISH
Muhammad Raa'fat Khalaf, Eman Mosaad Zaki, Abeer Moustafa Darwish, Mohamed Galal Mostafa El-Naggar
April-June 2015, 40(2):66-73
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.
  1,437 136 1
Prevalence of Helicobacter pylori infection among β-thalassemia major children with recurrent abdominal pain at Suez Canal University Hospital
GK Yousab Feiby, M Handoka Nesrin, AM Mesbah Badr
April-June 2015, 40(2):74-79
Background Recurrent abdominal pain (RAP) is a common pediatric problem and it is also one the several gastrointestinal tract disturbances frequently seen in β-thalassemia major (β-TM) patients. Helicobacter pylori is known to be an etiological agent of chronic gastritis and peptic ulcer diseases, but its correlation with RAP is still under debate. Aim The aim of the study was to determine the prevalence of H. pylori infection among β-TM children with RAP. Patients and methods This study was cross-sectional in design. We recruited 48 β-TM cases (24 β-TM children who presented with RAP and 24 β-TM children who did not present with RAP), aged 5-18 years, and 48 age-matched and sex-matched controls (24 children who presented with RAP without any other apparent manifestations of disease and 24 healthy children who did not present with RAP). Children who presented with RAP fulfilled Apley's criteria for the diagnosis of RAP. All patients and controls had undergone serum H. pylori IgG evaluation by ELISA. Results The seroprevalence of H. pylori in β-TM patients who presented with RAP was more common than in controls who presented with RAP [14/24 (58.3%) vs. 7/24 (29.2%); P = 0.04]. Also, there was a statistically significantly higher seroprevalence of H. pylori among the total number of β-TM children than among the total number of control children [24/48 (50%) vs. 12/48 (25%)]. The mean duration of splenectomy was statistically significantly higher in total β-TM children who were H. pylori IgG positive than among total β-TM children who were H. pylori IgG negative. There was no significant difference between total H. pylori IgG-positive β-TM children and age, sex, duration of blood transfusion, duration of iron chelating agent, criteria of abdominal pain, and serum ferritin level. Conclusion There was a higher prevalence of H. pylori IgG in β-TM children with RAP than among control children with RAP, as well as among total β-TM children than among total control children. H. pylori IgG was detected more frequently in thalassemic patients with a longer duration of splenectomy.
  1,307 191 -
Risk and prognostic factors for invasive fungal diseases in paediatric patients with malignancy
Fatma S. E. Ebeid, Rod Skinner
April-June 2015, 40(2):55-59
Background Invasive fungal diseases (IFDs) remain a challenge in the treatment of patients with haematological malignancies. Aims The aim of the study was to evaluate factors that are thought to favour the onset of IFDs and identify prognostic factors that could predict the eventual outcome of these infections. Patients and methods A systematic review of the literature was conducted for an explicit identification of risk and prognostics factors for IFDs in immunocompromised children. We retrospectively reviewed two age-matched and sex-matched patients with refractory acute myeloid leukaemia who had undergone allogeneic bone marrow transplantation. Results Both patients were exposed to the same hospital conditions and had been admitted for the first time at nearly the same period. They had two different paths as regards the development and outcome of IFD. Conclusion Several factors may have an impact on the onset and outcome of IFDs, and identification of these factors is essential to the evaluation and management of IFD and for timely establishment of appropriate antifungal therapy. Egyptian J Haematol 40:-0 ͹ 2015 The Egyptian Society of Haematology.
  1,115 140 -
The serum high-mobility group box 1 level and RAGE expression in childhood acute lymphoblastic leukemic patients'
Manal H Farahat, Mohammad A Sharaf, Tarek A Attia
April-June 2015, 40(2):60-65
Introduction High-mobility group box 1 (HMGB1) overexpression renders cancer cells resistant to apoptosis, and plays an important role in chemotherapy resistance. The aim of the present study was to measure serum high-mobility group box 1 (sHMGB1) and its receptor expression, advanced glycation end (RAGE) on childhood acute lymphoblastic leukemia (ALL) blast cells, and their correlation with different variables. Participants and methods Twenty-eight newly diagnosed childhood ALL cases (group I), 24 patients with childhood ALL in complete remission (group II), and 22 apparently normal individuals matched for age and sex as the control group (group III) were included in the study. In addition to routine laboratory investigations, sHMGB1 was measured by enzyme-linked immunosorbent assay, and RAGE expression on mononuclear cells from the bone marrow and/or the peripheral blood was assessed using monoclonal antibodies by flow cytometry for all participants. Results sHMGB1 and RAGE expression were significantly higher in ALL (group I) when compared with other groups; there was no significant difference between groups II and III with regard to sHMGB1 and RAGE expression. There were significant positive correlations between sHMGB1 and the white blood cell count and the bone marrow blast percentage in group I, and no significant correlations were found between sHMGB1, the RAGE expression, and other variables. Conclusion The present study showed significant upregulation of sHMGB1 in all cases and RAGE expression in a few cases of childhood ALL. HMGB1 may represent an important potential target for cancer therapeutics. Hence, we recommend the concomitant use of HMGB1-neutralizing antibodies and potential HMGB1 release inhibitors (e.g. quercetin) with the chemotherapeutic agents used in childhood ALL treatment.
  1,011 166 1
Immunoglobulin heavy-chain gene rearrangement in B-cell non-Hodgkin lymphoma using the fluorescence in-situ hybridization technique
Mohamed A. M. Mekawy, Manal M Ismail, Mahira I El Mogy, Ahmed M. M. Mostafa, Saad S Eissa, Samy A Abu Sikkien, Ghada M ElGohary
April-June 2015, 40(2):90-98
Objectives Non-Hodgkin lymphoma (NHL) comprises an extremely heterogeneous group of clonal lymphoproliferative disorders that might be derived from either B-cell or T/NK-cell lineages. The molecular pathogenesis of NHL represents a complex process involving the accumulation of multiple genetic lesions, which include the activation of proto-oncogens such as BCL-1, BCL2, BCL6, and c-MYC by chromosomal translocation, as well as inactivation of tumor-suppressor genes such as TP53 by chromosomal deletion or mutation. Aim of the study The present study aimed to detect immunoglobulin heavy-chain (IgH) gene rearrangement by the fluorescent in-situ hybridization (FISH) technique in paraffin-embedded bone marrow trephine and lymph node biopsies, and to correlate the presence of IgH chain gene rearrangement to the standard prognostic factors of NHL. Participants and methods The present study was carried out on 50 newly diagnosed adults with NHL. The study included 26 diffuse large B-cell lymphoma patients, 21 follicular lymphoma (FL) patients, and three mantle cell lymphoma patients. FISH was performed using LSI 14q break-apart rearrangement probes. Results The IgH gene rearrangement was detected by the FISH technique in lymph node sections of 36 out of 50 patients (72%) and only in six patients (12%) by trephine biopsy; also, it was found that 14q+ve patients were significantly associated with advanced stage of disease as well as low hemoglobin level, high total leukocytic count, low platelet count, high peripheral blood lymphocyte percentage, high lactate dehydrogenase level, and a high International Prognostic Index score. For diffuse large B-cell lymphoma, IgH gene rearrangement was detected in lymph node sections of 17/26 (65.4%) patients and in two patients by trephine biopsy, indicating a highly significant difference. In FL patients, IgH gene rearrangement was detected by the FISH technique in lymph node sections in 16/21 (76%) patients and in one patient (5%) by trephine biopsy, with a high statistical significance. For mantle cell lymphoma, IgH gene rearrangement was detected in all patients 3/3 (100%) both by lymph node and by trephine biopsies. Conclusion This study shows the increasing importance of detailed cytogenetic analysis of NHL cases and focuses on the necessity of use of the FISH technique on lymph node as it identifies early cytogenetic aberrations that are not detected in a bone marrow trephine biopsy, except in stage IV lymphoma.
  1,091 82 -
Thrombin-activatable fibrinolysis inhibitor antigen level in coronary artery disease
Hebatallah Adel Sedky, Deena Mohamed Mohamed Habashy, Walid Abdel Salam Ammar, Eman Fathy Mohamed El-Ashmawy
April-June 2015, 40(2):49-54
Background Thrombosis is one of the most serious complications associated with coronary artery disease (CAD), possibly arising from increased platelet activation and/or loss of the regulation of coagulation and fibrinolysis. Objectives We aimed to measure total thrombin-activatable fibrinolysis inhibitor antigen (t-TAFI Ag) level in CAD Egyptian patients and correlate its level with the standard risk factors for CAD, to investigate its possible role in the occurrence of atherothrombosis in those patients. Patients and methods Plasma t-TAFI Ag level was measured by enzyme-linked immunosorbent assay in 70 CAD patients and 20 healthy sex-matched and age-matched volunteers as a control group. Results Plasma t-TAFI Ag level was higher in CAD patients [both acute coronary syndrome and stable angina groups (groups I and II, respectively)] compared with the control group (P = 0.001), whereas no difference was found in t-TAFI Ag level, neither between group I and II (P = 0.37) nor between subgroups of acute coronary syndrome (P = 0.66). Regarding correlation with risk factors, t-TAFI Ag level was inversely correlated with high-density lipoproteins only in group I (P = 0.02). Conclusion t-TAFI Ag level is increased in CAD Egyptian patients and this increase may play an important role in the occurrence of thrombotic attacks in these patients.
  1,024 97 -
Expression of B-cell activating factor in acute lymphoblastic leukemia patients
Mona F Abd El Fatah, Emad A Abd El-hadi, Tahany A Helmy
April-June 2015, 40(2):99-103
Introduction Acute lymphoblastic leukemia (ALL) is a biologically heterogeneous disorder needing determination of morphologic, immunologic, cytogenetic, biochemical, and molecular genetics characterizations of lymphoblasts to establish diagnosis. Microenvironmental cues play critical roles in cancer biology and malignant cells are responsive to multiple extrinsic factors. There is emerging evidence that B-cell activating factor (BAFF) is a critical factor for the growth and survival of both normal and malignant clones of B-cells and can augment tumor cell growth. There is evidence that B-lineage neoplasms have aberrant expression of BAFF. Objectives The aim of this study was to evaluate serum BAFF levels to assess its prognostic impact on ALL and correlate it with mortality. Patients and methods The study was conducted at Ain Shams University on 40 ALL pediatric and adult patients, in addition to 20 controls. Patients underwent detailed history and clinical examination, bone marrow examination, and evaluation of serum BAFF levels using enzyme-linked immunosorbent assay. Thereafter, they were assessed for treatment response and patients' mortality on day 28 from induction of chemotherapy and after 6 months, respectively. Results We found a statistically significant lower BAFF levels among controls compared with the patient group, in which there was no significant difference between pediatric and adult subgroups. High serum BAFF was significantly correlated to patients' poor treatment response but not mortality. Conclusion High BAFF in our studied patients may be explained by its role in augmenting B-cell tumor growth. In our study, we found that BAFF had no relation with disease outcome. This may be caused by the limited number of patients included. Assessment of BAFF level at the time of diagnosis may be a predictor for response to treatment, as we found a significant relation between BAFF level and response to treatment. This finding recommends that patients with high BAFF level at the time of diagnosis be subjected to intensified course of therapy.
  886 82 -
Fludarabine and bradycardia in recipients of allogeneic stem cell transplant: a case series
Chepsy C Philip
April-June 2015, 40(2):104-106
Fludarabine is being increasingly used both as a chemoimmunotherapeutic agent and in conditioning regimens for stem cell transplant. ECG disturbances with this increasingly used drug have never been reported. This report describes a patient who developed bradycardia following fludarabine administration, prompting an assessment for rhythm irregularities in subsequent patients undergoing stem cell transplant using a fludarabine-based conditioning regimen. In a series of 10 patients who received fludarabine as part of their conditioning, there was a significant effect on QTc interval (mean difference 30.6 ms; P = 0.002) and heart rate (mean difference −18.9 beats/min; P = 0.003). In conclusion, caregivers need to be alert to recognize the potential for unrecognized adverse events even in frequently used drugs. Fludarabine has a potential for rhythm disturbances.
  894 70 -
Prognostic impact of neuropilin-1 expression in children with B-lineage acute lymphoblastic leukemia: Retraction

April-June 2015, 40(2):107-107
  635 64 -