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Table of Contents
January-June 2013
Volume 2 | Issue 1
Page Nos. 1-51
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ARTICLE
Non- Hodgkin's Lymphoma; Epidemiology and Presentation
p. 1
Zead Ibrahim, Allawi NHussein, AbdulSattar I AL-Kubaysi, Tariq A AL-Shujairi, Jaffar M AL-Gabban
Background:
The non-Hodgkin lymphomas (NHL) of childhood are a diverse collection of diseases originating in the cells and organs of immune system.
Objectives:
To determine epidemiology and clinicopathological presentations of children with NHL admitted to Child's Central Teaching Hospital.
Methods:
A retrospective study was done from 1st of January 2004 to the 31st of December 2009; the patients with newly diagnosed NHL, age less than 15 years, who were admitted to the pediatric oncology unit in the Child's Central Teaching Hospital.
Results:
The total number of patients was 84; the mean age at diagnosis was 6.3years, with a male to female ratio of 2:1. Most of patients were presented in stage III&IV (88%).Most common presenting features were abdominal distension or a mass in 51%.Burkitt lymphoma and Burkitt like lymphoma were the most common histological subtype (58.33%).
Conclusions:
The majority of cases were between 5-9 year age group, and the mean age at presentation was 6.3 years old, with a male to female ratio of 2:1. The most common presenting site was the abdomen. The majority of cases were fallen in advanced stages (III&IV). Histopathologically Burkitt's lymphoma was the commonest subtype.
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Assessment of GM-CSF Level in the Serum of Patients with Different Stages of Chronic Myeloid Leukemia Befor and After Imatinb Mesylate Therapy
p. 7
Shahla'a Fadhil Sabir, Maysoon Ali Saleem, Bassam Francis Matti
Background:
Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of primitive haemopoietic progenitor cells. The cytogenetic hallmark of CML is the Philadelphia chromosome, created by a reciprocal translocation between chromosomes 9&22 (t [9;22][q34;q11]). Survival and amplification of hematopoietic progenitors are controlled by a number of regulatory molecules (hematopoietic growth factors). The role of Granulocyte- macrophage colony-stimulating factor (GM-CSF) and its receptor in pathology arises largely as a result of abnormal signaling leading to deregulated myelopoiesis. The development of the BCR-ABL-targeted Imatinib mesylate represents a paradigm shift in the treatment of CML. Data indicate that the level of immune responses against CML is low before Imatinib, rises as treatment is administered and declines again when the BCR-ABL transcript numbers fall to low levels.
Objective:
To assess and evaluate the significance of levels difference in GM-CSF through newly diagnosed patients, different responder groups (optimal, suboptimal and failure cytogenetic response) and advanced stages (acceleration and crisis groups) of CML Iraqi patients whom receiving Imatinib mesylate (TKI), as an indicator to assess the role of growth factor in pathogenesis of CML disease development and response to treatments.
Patients and methods:
In this study 128 Iraqi CML patients asses before and after receiving imatinib mesylate treatment which categorized by complete blood picture and fluorescent
in situ
hybridization (FISH) analysis in to different response groups and stages, then used an ELISA technique to assess serum level of GM-CSF in each response group and advance stage (acceleration and transformed) of CML patients, in comparison to level in 32 healthy control subjects.
Results
: Out of 128 patients the mean of GM-CSF serum level (pg/ml) for the newly diagnosed, optimal responded, suboptimal responded, failure cytogenetic and advance stage of CML were 399.53±104.50, 325.23±66.37, 428.90±45.70, 347.12±54.45, 521.56±83.73, respectively. While healthy was 269.25±86.27.
Conclusion:
Uncontrolled granulopoiesis in newly diagnosed patients with CML may be mediated by increased plasma CSF concentrations caused by BCR-ABL activity which may give an idea regarding the role of colony stimulating factors in the pathogenesis of CML disease.
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Gender Disparity in Clinical Presentation, Immunophenotype, and Early Steroid Response in Pediatric Acute Lymphoblastic Leukemia Patients
p. 14
Balsam Fadhil Abid Salih, Subh Salem Al-Mudallel, Sajed Saad Mohammed
Background:
Acute leukemia is the most common type of childhood cancer, of which acute lymphocytic leukemia (ALL) comprises 78% of cases. Incidence rate, prognosis and survival of childhood ALL patients differ according to gender. Despite overall improvements in survival of children with ALL, male children still experience poorer survival.
Objective
is to explore differences between male and female pediatric patients with newly diagnosed ALL regarding their presenting clinical features, ALL immunophenotype, and early steroid response.
Methods:
This study was prospectively designed to include 60 newly diagnosed pediatric ALL patients from April 2011 to March 2013. Each patient was assessed clinically at admission and at the end of a 7-day prednisone prophase to be classified as a prednisone-good responder (<1000/μL peripheral blood blasts on day 8) or a prednisone-poor responder (>1000/μL). Immunophenotype was determined by immunocytochemical staining of bone marrow aspirates for cCD79a (specific for B-cells) and cCD3 (specific for T-cells).
Results:
The study group consisted of 38 males and 22 females. The median age was 62.5 months for males and 41.5 months for females. Splenomegaly was found in 71% of males versus 63.6% of females, hepatomegaly in 68.4% of males versus 45.5% of females, mediastinal masses were detected in 6 males and 3 females, and CNS disease affected 5 patients, 3 males and 2 females (
p
>0.05). WBC mean count was 63.78±15.98 x109/L in males and 49.2±21.87 x109/L in females, the mean Hb was 8.75±0.53 g/dl in males and 7.91±0.34 g/dl in females (
p
>0.05). 75.8% of male patients were B-ALL and 24.3% were T-ALL, and 76.5% of females were B- ALL and 23.5% were T-ALL. 86.8% of male patients and 86.4% of female patients were good steroid responders (
p
> 0.05).
Conclusions:
Pediatric male patients were more frequent and older than females, and presented with clinical and hematological features considered to be of poor prognosis more than females. No significant difference was observed regarding ALL immunophenotypes and early steroid response.
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Expression of CD7, CD13, CD14, CD33 and CD34 on Myeloblasts in Bone Marrow Aspirate of Patients with Newly Diagnosed Acute Myeloid Leukemia Using Multicolor Flow Cytometry
p. 27
Abbas H Abdulsalam, Subh S Al-Mudallal, Ghassan A Khaleel
Background:
An important part of diagnosis of acute leukemia is to decide on the lineage whether it is of myeloid or lymphoid. Familiarity with expression of various surface antigen markers is essential to diagnose difficult cases and to follow up for minimum residual disease.
Aim of the study:
To evaluate the expression of CD7, CD13, CD14, CD33 and CD34 in bone marrow aspirate of patients with newly diagnosed AML using multicolor multiparametric flow cytometry.
Patients, materials and methods:
This is a prospective study which includes 21 newly diagnosed adult patients with AML from April 2012 to March 2013. Flow cytometry analysis for CD7, CD13, CD14, CD33 and CD34 was carried out on bone marrow aspirate samples using 2-laser, 4-color PARTEC Cube6 and using De Novo FCS Express 4 Flow Cytometry software. The sensitivity of fluorescent detectors was monitored using standard beads according to the manufacturer's recommendations and normal lymphoid cells within specimens served as internal positive and negative controls. Only samples with blasts that contain Auer rods, positive for SBB cytochemical stain (≥3%) and/or MPO+ by FC (≥10%) are included.
Results:
The median, range and interquartile range percentages for myeloblasts, CD7, CD13, CD14, CD33 and CD34 are 48, 22-84 and 24; 30, 20-36 and 7; 62, 34-98 and 28; 53, 21-86 and 6; 56, 30-91 and 17; and 73, 25-94 and 34respectively.
Conclusions:
The use of FC at diagnosis of AML can be useful to add objective confirmation especially in cases where Auer rods are not present and when SBB stain did not add towards the diagnosis. Moreover, if the aim is to follow up for MRD then the use of FC becomes essential.
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Type 2 Diabetes Mellitus is causing red blood cell hemolysis
p. 32
Zainab Mohammed Hasan
Background:
Diabetes mellitus is a metabolic disease and its complications are interlinked and usually have a common soil. The clinical effect of diabetes on erythropoiesis is always interesting as the production of red cells is always at high rate and continuous, yet the red cell mean life span of 120 days is of an appropriate length, making it an excellent candidate for tests to detect the effect of diabetes and its complications and monitor for the response to treatment.
Aim of the study:
To determine the level of reticulocyte percent in patients with uncomplicated type 2 Diabetes Mellitus and to evaluate the effect of folic acid treatment on this percent.
Patients, controls and methods:
140 patients with type 2 Diabetes Mellitus were included in this study. The exclusion criteria from this study were anemia, pregnancy, personal and family history of hemolytic anemia, overt microvascular complications of DM (retinopathy, neuropathy and nephropathy), acute infection and/or inflammation and history of chronic disease other than diabetes. The patient medical record was reviewed and peripheral blood specimen withdrawn for determination of hemoglobin concentration, PCV %, reticulocyte % and HbA1c. A subgroup of (82) patients (45 males and 37 females) were chosen who consented on not to change their treatment for the next coming one month except for the addition of daily one 5mg tablet of folic acid. PCV %, hemoglobin concentration, reticulocytes % were tested for using manual techniques. HbA1c % was measured using automated HPLC machine.
Results:
This study revealed increased red cell destruction in type 2 diabetics in comparison to healthy control subjects of the same sex and age. Also the reticulocyte increment was more in those with higher HbA1c %, although it was not in linear relationship with it. These findings are suggesting that the initiating event for red cell hemolysis is the increased blood glucose level.
Conclusion:
Type 2 diabetes patients are subject to oxidative stress as a result of hyperglycemia. This study suggests that the addition of folic acid treatment to the regime of type 2 diabetic patients can be useful.
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Incidence of Von Willebrand Disease Among Patients presenting with Various Bleeding Tendency to Out-Patient Clinic of the National Center of Hematology
p. 37
Alaa Fadhil Alwan, Zeyad Ahmed Shabeeb, Hadeel duraid salman
Background
: Von Willebrand disease is frequent hereditary bleeding disorders with an incidence of about 1% in asymptomatic people. Previous Studies available around the Middle East displayed a prevalence ranged from 3 % to 34 % of Von Willebrand disease within the hereditary bleeding disorders. People with mucocutaneous bleeding represent a major subtype of hematologic clinical presentations but simultaneously present a substantial diagnostic challenge. On the other hand, bleeding symptoms are frequent in the general population, but their clinical relevance may be difficult to assess The aim of this study was to estimate the incidence of Von Willebrand disease in patients presenting with various bleeding tendencies to out-patient clinic of the national center of hematology
Methods:
A total of 146 sequential patients referred to the national center of hematology between January 2011 and April 2012 were investigated .Tests performed for the diagnosis of Von Willebrand disease included complete blood count and blood film including platelet count, bleeding time, prothrombin time, activated partial thromboplastin time, Factor VIII:C assay, and von Willebrand Factor Antigen assay.
Results
: Amongst 146 patients, 29 (19.8%) had Von Willebrand disease. Patients' age ranged from 1 year to 65 years, with 35 males and 111 females. Menorrhagia was the most common presentation. Amongst vWD patients, there were 7 male and 22 female. Positive family history in patients with VWD was found in 21 out of 29 patients (72.4 %) while positive family history in bleeding tendency other than VWD was found in 47 patients (40%). Statistical significant differences were found in prothrombin time, activated partial thromboplastin time, Factor VIII: C assay, and von Willebrand Factor Antigen assay between the studied groups.
Conclusions:
Von Willebrand disease still among the most common cause of inherited bleeding tendency in patients presented with mucocutaneous or menorrhagia, yet many cases of vWD remain undiagnosed due to wide range of clinical presentations and lack in lab diagnosis.
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Pleural Effusion as A Manifestation of Extramedullary Blastic Crisis in A Patient with Chronic Myeloid Leukaemia
p. 43
Adel S Al-Aqabi, Hassanain H Hassan, Farah A Hussein, Mustafa N Abd Ali
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LETTER TO EDITOR
Incidence of hemoglobinopathies among anemic patients visiting National Center of Hematology
p. 48
Abdulsalam Hatim Mohamed, Jawad kadum Mshali, Abeer Mohamed
The hemoglobinopathies encompass heterogeneous group of disorders associated with either single gene mutation in α globin and β globin genes (as in α and β thalassemia).the aim was to estimate the incidence of hemoglobinopathies in patients with hypochromic microcytic anemia during the period from 1st January 2011 till 31st December 2012 at the National Center of Hematology ion a total of 1103 patients by using high performance liquid chromatography (HPLC) technique. we found these results: 656 patients (58.9928%) shows normal Hb-electrophoreses while 447 patients (40.5258%)shows abnormal Hb-electrophoresis
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