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Table of Contents
July-December 2020
Volume 9 | Issue 2
Page Nos. 55-171
Online since Tuesday, November 10, 2020
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REVIEW ARTICLE
Hereditary hemorrhagic telangiectasia: An informative review
p. 55
Neha Rajpurohit, Piyush Kumar Bharbey, M Jatin, Khayati Moudgil
DOI
:10.4103/ijh.ijh_24_20
Inherited hemorrhagic telangiectasia (HHT or Osler–Weber–Rendu syndrome) is a hereditary condition characterized by malformations of multiple blood vessels (vascular dysplasia), which may lead to bleeding (hemorrhaging). Chronic nosebleeds are often the first warning, and malformations in various blood vessels can lead to abnormalities in the lungs, brain, spinal cord, and liver. There are a number of therapies available for various aspects of HHT to improve the quality of life and avoid life-threatening complications. Individuals with HHT have an almost average life expectancy. HHT is inherited as a dominant autosomal trait. We have done this review to enlighten the scientific fraternity about HHT. In this review, we have tried to explain about HHT and its related management.
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ORIGINAL ARTICLES
Growth-differentiation factor-15 expression in anemia of chronic disease and iron-deficiency anemia
p. 61
Noor Sabeeh Abdullah, Abeer Anwer Ahmed
DOI
:10.4103/ijh.ijh_7_20
BACKGROUND:
Iron-deficiency anemia (IDA) is a common hematological disorder. Anemia of chronic disease (ACD) is mild-to-moderately severe anemia associated with chronic diseases. Growth-differentiation factor-15 (GDF-15) is a member of the transforming growth factor-
β
, produced by late-stage erythroid precursors in the bone marrow; it suppresses hepcidin expression during ineffective erythropoiesis. The aim of this study was to measure the level of the GDF-15 level in ACD and IDA and to evaluate its ability to differentiate between ACD and ACD with coexisting iron deficiency (mixed anemia).
PATIENTS, MATERIALS AND METHODS:
The present study including 87 persons “30 with IDA, 30 with ACD, and 27 controls.” The following investigations were done: complete blood count, erythrocyte sedimentation rate, iron profile (serum iron and total iron-binding capacity), C-reactive protein, ferritin, hepcidin, and GDF-15, depending on the results of these investigations, some patients of ACD group appear to be ACD with co-existing iron deficiency. The analysis of data was carried out using the Statistical Packages for the Social Sciences software version 24.
RESULTS:
The serum hepcidin was significantly lower in IDA, whereas the serum GDF-15 is comparable to that of the control group. The serum hepcidin and serum GDF-15 were significantly higher in (ACD and mixed anemia) group compared to the control group.
CONCLUSION:
GDF-15 is high in ACD group and comparable to the control in iron-deficiency group, and it is not a useful marker to differentiate between ACD and ACD with coexisting iron deficiency.
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High-density lipoprotein functionality and AB blood phenotype
p. 66
Esin Eren, Necat Yılmaz, Belkıs Koctekin, Suayip Birinci
DOI
:10.4103/ijh.ijh_10_20
BACKGROUND:
The impact of the different ABO blood phenotypes has not been extensively investigated on high-density lipoprotein (HDL) functionality. Therefore, the aim of this study was to assess HDL-related serum paraoxonase 1 (PON1), ApolipoproteinA-1 (ApoA-I) and also oxidized low-density lipoprotein (OxLDL) in relation to ABO blood groups.
SUBJECTS AND METHODS:
ABO blood types represent part of the genetic phenotype. Therefore, we assume that ABO blood phenotypes are associated with many diseases. This cross-sectional study involved 169 apparently healthy male medical staff with different ABO blood phenotypes.
RESULTS:
The most important of the findings in this study is that the amount of PON1, which is one of the valuable markers of HDL function, was statistically significantly lower in people with AB blood group than in other blood groups. Furthermore, one notable important findings of this study are the lowest serum ApoA-I levels of individuals in AB blood phenotype. On the contrary, serum OxLDL levels in the subjects with AB blood phenotype are increased.
CONCLUSION:
There is no doubt that it is necessary to examine in more detail the molecular changes in HDL metabolism linked to the ABO blood group phenotype. What is really interesting is that the AB blood phenotype in many Mediterranean countries has the lowest rate of distribution. According to the findings from this study, the A and B blood group phenotypes may be more effective than expected in disease susceptibility.
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Renal phosphate loss in Iraqi chronic myeloid leukemia patients treated by imatinib mesylate
p. 72
Huda A Al-Taee, Azhar K Athab, Ala Ali
DOI
:10.4103/ijh.ijh_11_20
BACKGROUND:
The use of BCR-ABL tyrosine kinase inhibitor imatinib mesylate improved outcomes for patients with chronic myeloid leukemia (CML). Hypophosphatemia is found to be associated with imatinib mesylate use; the exact mechanism of this is not clear yet but mostly related to a drug-induced proximal renal tubular defect.
OBJECTIVE:
The objective was to measure the renal phosphate loss in CML patients treated by imatinib mesylate.
PATIENTS AND METHODS:
A cross-sectional study included 40 patients (25 females) who were already diagnosed cases and treated with imatinib mesylate (400 mg/day). The mean age was 40.2 ± 7.8 years. The study was conducted at the medical city teaching hospital, Baghdad, hematology outpatient clinic from July 2016 to December 2016. Serum and random urine samples were measured phosphate and creatinine in serum and urine, respectively. Serum and urine phosphate were measured using the colorimetric method, whereas serum and urine creatinine were calculated by the kinetic method. Fractional excretion of phosphate and tubular maximum of phosphate reabsorption were calculated. After the completion of the study, we tested ten newly diagnosed patients at 0- and 3 months of treatment.
RESULTS:
Sixteen patients (40%) developed hypophosphatemia. The fractional excretion of phosphate increased (FEPO4 = 21.1%) with a comparable reduction in tubular reabsorption of phosphate to the glomerular filtration rate (Tmpi/GFR = 2.3 mg/dl). There was a significant direct correlation between SPO4 level and white blood cell count (R = 0.451;
P
= 0.001). The mean intact parathyroid hormone and Vitamin D levels were normal for the study group. All ten newly diagnosed cases developed hypophosphatemia at 3 months. This was statistically significant (
P
= 0.002). There increase in FEPO4 and decrease in Tmpi/GFR was statistically significant (
P
< 0.001 and 0.002), respectively.
CONCLUSION:
Hypophosphatemia while using imatinib mesylate is due to increased urinary phosphate excretion.
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Diagnosis of patients with hemoglobinopathies including α-thalassemia in a laboratory with limited resources
p. 77
Abbas Hashim Abdulsalam, Subh Salim Al-Mudallal, Nidhal Karim Al-Rahal
DOI
:10.4103/ijh.ijh_12_20
BACKGROUND:
Diagnosis of α-thalassemia can be challenging as it is clinically insignificant in the majority of patients who are presented with one or two α-gene deletion, it cannot be always suspected from the red cell indices, and the confirmatory tests of α-thalassemia are not readily available, applied, and/or provide conclusive results. The importance of diagnosis of α-thalassemia is mainly to identify the patients with α
0
-thalassemia, hemoglobin (Hb) H disease, Hb Bart's hydrops fetalis, and to identify the ameliorating effect that the coinheritance of α-thalassemia may have on the clinical phenotype of patients with HbS and other structural β-globin chain variants and/or patients with beta-thalassemia. The aim of this study is to evaluate the presentation and diagnosis of patients with α-thalassemia in a cohort of Iraqi patients with hemoglobinopathies.
PATIENTS, MATERIALS, AND METHODS:
This is a prospective cohort study that included 216 patients diagnosed with different types of hemoglobinopathies from June 2016 to October 2019. For each patient after reviewing the cause of presentation and family history of thalassemia, blood samples were sent for complete blood count, including calculated absolute reticulocyte count, peripheral blood smear, Hb H preparation, serum iron and total iron-binding capacity, sickling test, and if indicated Hb high-performance liquid chromatography (HPLC) (using BioRad D10). Family study was requested for several patients.
RESULTS:
Male:female for patients with α-thalassemia is 1:1.25. Consanguinity and family history of α-thalassemia are present in 85.2% and 51.9%, respectively. Out of 216 patients with different types of hemoglobinopathies, 25% are diagnosed with isolated or copresence of α-thalassemia. Low/lower normal HbA
2
% is the most sensitive parameter for the diagnosis of α-thalassemia.
CONCLUSIONS:
The most sensitive parameter for the diagnosis of α-thalassemia in this study is the low/lower normal HbA
2
% in patient with normal iron study. Other parameters such as the presence of golf-ball red cells in Hb H preparation and/or presence of HbH wave in Hb HPLC can be helpful but are only seen in smaller fraction of patients.
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Evaluation of interleukin-35 and interleukin-10 in adult acute myeloid leukemia patients before and after induction chemotherapy
p. 82
Eman Fadhil Mahmood, Abeer Anwer Ahmed
DOI
:10.4103/ijh.ijh_17_20
BACKGROUND:
Acute myeloid leukemia (AML) is a clonal proliferation of hemopoietic cells. Interleukin-35 (IL-35) is a pro-inflammatory cytokine expressed in T regulatory (Treg) and B regulatory cells. IL-35 promotes the proliferation of AML blasts and lessens apoptosis. Hence, IL-35-derived from Tregs promotes the growth of adult AML blasts, suggesting that IL-35 has an important role in the pathogenesis of AML. IL-10 is anti-inflammatory cytokines with immune-stimulatory activities and is formed by CD4 and CD8 T-cells and activated B-lymphocytes.
OBJECTIVES:
The aim of th study was to estimate the levels of IL-10 and IL-35 in the sera of patients with AML before and after chemotherapy induction and to correlate levels with blast cells percentage and other hematological parameters.
PATIENTS, MATERIALS AND METHODS:
This study was conducted on thirty newly diagnosed (ND),
de novo
adult AML patients, 15 males and 15 females with a age range between 19 and 75 years for a period from September 20, 2017, to March 15, 2018. It included 18 healthy (9 males and 9 females) individuals who were taken as a control group. Diagnosis of AML was established according to the morphology, cytochemistry and flow cytometry study of both peripheral blood and bone marrow aspiration as well as biopsy reports. ILs-35 and 10 levels were measured at diagnosis and after induction chemotherapy when achieving complete remission based on Cheson
et al
. definition.
RESULTS:
Serum IL-35 and IL-10 were significantly higher in ND AML patients than controls and were reduced after induction chemotherapy (
P
< 0.001). In patients with remission, IL-10 was significantly reduced compared with non-remission group, while the reduction in IL-35 level in remitted compared to nonremitted patients did not reach the level of significance (
P
> 0.001). No correlations were found between hemoglobin, platelet counts, white cell count, and blast percentage with IL-35 and 10 levels in
de novo
AML patients.
CONCLUSIONS:
Both ILs-35 and 10 levels were elevated in
de novo
AML patients and lowered following induction chemotherapy. Both ILs have no correlation with hemoglobin level, platelet count, white blood cell count, and blast percentage.
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Effect of remission induction therapy on the level of soluble urokinase plasminogen activator receptor in acute myeloid leukemia
p. 87
Eman Qassim Ahmed, Haithem Ahmed Al-Rubaie
DOI
:10.4103/ijh.ijh_20_20
BACKGROUND:
Soluble urokinase plasminogen activator receptor (suPAR) in acute myeloid leukemia (AML) is associated with resistance to chemotherapy. The aim is to assess the effect of remission induction on plasma level of suPAR in AML patients and to correlate between plasma levels of suPAR with certain hematological parameters, AML subtypes, and extramedullary involvement.
MATERIALS AND METHODS:
The study was conducted on 30 newly diagnosed adult AML patients and 20 controls. The peripheral blood and bone marrow smears were examined at diagnosis and after remission induction therapy to assess complete remission (CR). The plasma level of suPAR was measured by enzyme immunoassay which was done at diagnosis and after remission induction therapy.
RESULTS:
Plasma suPAR level was higher in AML patients at presentation than the control group (
P
< 0.001). The plasma level of suPAR revealed a significant reduction in the 37% of patients who achieved CR or partial response (
P
= 0.004), whereas the differences were insignificant and remained high in patients who did not show response (
P
= 0.573). Before and after treatment, there were insignificant differences in suPAR level in patients with or without extramedullary involvement and in various AML subgroups (
P
> 0.05). The suPAR levels were high before starting treatment in both the alive and those who deceased (
P
= 0.984). After 5 months of follow-up, it showed a significant reduction among the alive group (
P
= 0.001). There were insignificant correlations between the level of uPAR and hematological parameters at presentation.
CONCLUSIONS:
The reduction of plasma suPAR level is associated with a better response, and a high level is associated with a high risk of death. Before and after induction therapy, there is no association between plasma level of suPAR in monocytic group, nonmonocytic group, and in patients with or without extramedullary involvement. Plasma suPAR level is uncorrelated with hematological parameters at presentation.
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Efficacy and safety of romiplostim in adult Iraqi patients with refractory immune thrombocytopenia
p. 92
Ali Khazaal Jumaa, Tareq Abdullah Saleh, Asaad Abdulameer Khalaf, Mohammed Saleem Abbas
DOI
:10.4103/ijh.ijh_21_20
BACKGROUND:
Immune thrombocytopenia (ITP) is an immune-mediated acquired disease characterized by decrease of the platelet count. The majority of adult patients progress to chronic stage with some of them failed to respond or relapsed after the second-line therapy. Romiplostim is a thrombopoietin receptor agonist recently approved for patients with chronic ITP, it was recently licensed and introduced for use in Iraq. The aim of this study was to assess the efficacy and safety of romiplostim among patients with refractory ITP.
PATIENTS AND METHODS:
This prospective study was conducted between April 2017 and June 2018 in two hematology centers in Baghdad and Basra. A group of 56 adult patients with refractory ITP enrolled in this study. The patients were evaluated before the weekly scheduled romiplostim and followed for efficacy and safety of the treatment.
RESULTS:
the median age was 39 years with a female-to-male ratio of 4:1. The response rate (platelet ≥50 × 10
9
/l) was observed in 75% of the enrolled patient. The time to initial response was 2.5 weeks, and the mean dose of romiplostim used to achieve a sustained response was 3.1 mcg/kg. The response rate was significantly higher among splenectomized (94.4% vs. 65.8%). There was no difference in response regarding the age and gender. The most frequent adverse effects were joint pain (35.7%), followed by headache (32.1%) and fatigue (21.4%), resolved spontaneously within 2 days. Six patients developed thrombocytosis without any thrombotic event.
CONCLUSIONS:
Romiplostim is an effective option among Iraqi patients with refractory ITP as a long-term treatment or as a bridge for another intervention, with a relatively safe toxicity profile.
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Prevalence of anemia among pediatric critical care survivors and impact of restrictive transfusion strategy on it: A study from North India
p. 97
Manideepa Maji, Saikat Mandal, Nowneet Kumar Bhat, Gita Negi, Sarika Agarwal
DOI
:10.4103/ijh.ijh_19_20
BACKGROUND:
Anemia occurs frequently in critically ill patients and it can impact on long-term outcome more so after a critical illness. Very little data are available about anemia in pediatric critical care survivors. Recent randomized control trials in children support the use of restrictive transfusion strategy in critically ill children.
OBJECTIVES:
This study aims to estimate the prevalence of anemia in pediatric critical care survivors, its causative factors and impact of restrictive transfusion strategy on its resolution.
PATIENTS AND METHODS:
In this retrospective observational study, patients who got discharged from pediatric intensive care unit (PICU), their clinical course, serial hemoglobin (Hb) level, and transfusion history were collected. Patients were divided into two groups according to transfusion strategy (restrictive and liberal group). Patients with anemia were followed up and persistence of anemia was noted monthly.
RESULTS:
In 54 cases enrolled in the study, 35 children had anemia (prevalence 35/54 = 64.8%). Statically significant difference between anemic and nonanemic groups was found in terms of duration of PICU stay and development of infection and no significant difference in age, gender, diagnosis, and requirement of mechanical ventilation. Among the 35 anemic children, restrictive transfusion strategy was followed in 21 and in 14 children liberal transfusion strategy was followed. Ten children were lost to follow-up. Anemia got resolved in 18 children (18/25 = 72%) and 7 children (7/25 = 20%) had persistent anemia. Among 25 children, 4 children in restrictive group (4/15 = 26.6%) and 3 children in liberal group (3/10 = 30%) had persistent anemia (statistically not significant;
P
> 0.05).
CONCLUSIONS:
A large proportion of PICU survivor children is anemic at discharge, this could be due to long duration PICU stay or acquired infection during hospital stay or following restrictive transfusion strategy but on a long-term follow-up no statistically significant difference was noted between two groups.
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Health-related quality of life in multiple myeloma in Kurdistan Iraq
p. 101
Ameer I. A. Badi, Nasir A Al-Allawi, Ahmed K Yassin, Banaz M Safar, Basil K Abdulla, Rawand P Shamoon, Truska A Amin, Zeki A Mohamed, Ali I Mohammed, Diveen J Hussein, Kawa M Hasan, Nawsherwan S Mohammed, Rezhin N Rajab, Friad Hiwaizi, Kanar J Karim, Abid M Hassan, Hisham A Getta, Najmaddin S. H. Khoshnaw, Sana D. Jalal, Akram M Mohammed, Dana A Abdullah
DOI
:10.4103/ijh.ijh_22_20
BACKGROUND:
Health-related quality of life (HRQoL) in multiple myeloma (MM) gained increasing importance to ensure that the improved survival is associated with improved life quality.
OBJECTIVES:
The aim of this study was to assess the HRQoL in a cohort of myeloma patients from Kurdistan region in Iraq.
MATERIALS AND METHODS:
This observational, cross-sectional, multi-center study enrolled 138 patients with symptomatic MM patients. The patients' records were retrieved and they were also clinically assessed and appropriately investigated at the time of enrolment. HRQoL was assessed using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Multiple Myeloma module (EROTC QLQ-MY20.
RESULTS:
The 138 patients included had a mean age of 60.4 years and included 83 males and 55 females. Significant predictors of worse HRQoL disease symptoms and side effects of treatment domains included increasing age, female sex, and increase number of treatment courses. Other predictors associated with worse disease symptom domain include presence of bone lesions and nonsecretory myeloma versus light chain myeloma. Basic parameters including the concentration of monoclonal band, hemoglobin, serum creatinine, calcium, and albumin were not significant associated with scores in any of the domain while serum LDH was associated with worse side effects of treatment scores. Furthermore, it was noted that patients who had underwent autologous stem cell transplants had better HRQoL in all domains compared to other modalities of therapy, though this did not reach significance.
CONCLUSIONS:
HRQoL in MM treated in Iraqi Kurdistan is not much different from their Western counterparts and several predictors of worse QoL were identified in this cohort of patients.
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Magnetic resonance imaging versus radiological skeletal survey of the lumbosacral spine in patients with advanced multiple myeloma: A single-institute experience
p. 107
Najmaddin S. H. Khoshnaw, Kawa A Mahmood, Ahmed K Yassin, Sana D Jalal, Hangaw A Qadir, Ali I Mohammed, Layth Mula-Hussain
DOI
:10.4103/ijh.ijh_23_20
BACKGROUND:
Multiple myeloma (MM) is a plasma cell malignancy in the bone marrow (BM), where imaging is an essential tool in its management.
OBJECTIVE:
The aim of this study was to compare the sensitivity of radiological skeletal survey (RSS) with magnetic resonance imaging (MRI) of the lumbosacral spine (LSS) in advanced MM patients.
PATIENTS AND METHODS:
We retrospectively reviewed the RSS and MRI of the LSS for 33 patients with a new diagnosis of symptomatic MM. Chi-squire test was used for comparing the results.
RESULTS:
Of 33 patients, 20 (60%) were male and 13 (40%) female, with a mean age of 61 years. Characteristic findings on RSS were osteopenia (86%), compression fractures (60.5%), multiple lytic lesions (39.5%), solitary focal lesion (6.06%), and normal findings (9.3%), The commonest findings on the MRI were combined diffuse and focal lesions (27.27%), multiple focal lesions of macronodular pattern (18.2%) and variegated (micronodular) pattern (15.15%), The other finding on MRI were diffuse homogeneous infiltration with SI> adjacent disc (12.12%) while SI ≤ adjacent disc (6.06%), solitary focal lesions (6.06%), and normal findings (15.15%). A majority (58.1%) of patients presented with an advanced stage and the pathological compression fracture found in 60% of cases. Focal lesions were detected in five patients (15.15%) whose radiographs were negative, and more lesions were detected in 11 patients (33.33%) with positive radiographs. Diffuse infiltration pattern was found in eight cases (24%) on MRI imaging when radiographs showed only osteopenia and in one patient (3%) in whom radiographs were normal.
CONCLUSIONS:
MRI had higher sensitivity in the detection of diffuse BM involvement and discovery of focal spinal lesions compared to conventional radiographs with a positive
P
value.
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Seasonality in acute promyelocytic leukemia: Fact or myth?
p. 113
Amer Shareef Mohammed, Talib Hassan Ali, Alaa Fadil Alwan
DOI
:10.4103/ijh.ijh_27_20
BACKGROUND:
Acute promyelocytic leukemia (APL) is a biologically and clinically distinct subtype of acute myeloid leukemia (AML) Etiological and seasonal factors that play a role in APL still unclear.
OBJECTIVES:
The aim of the study was to retrospectively evaluate the available data to assess the impact of seasonal variations on incidence of APL over a period of 6 years.
PATIENTS AND METHODS:
A retrospective, observational study based on APL record of a Hematology Nasiriyah Center from January 2014 to January 2020 was processed. The collected data of diagnosed (APL) patients at this center for 6 years was analyzed, and APL presenting in each month of the year was also assessed for any evidence of seasonality.
RESULTS:
Fifty-eight cases of APL were included in this study: there were 22 males (37.93%) and 36 females (62.07%). The mean age was 31.1 ± 14.4 years (minimum 15 and maximum 67). We found a pronounced peak of APL occurrence in March (within winter and early spring months) and decline in summer to zero point in August, which was repeated periodical all studied 6 years.
CONCLUSIONS:
Seasonal pattern of APL was observed. Investigation of specific seasonal risk factors would be informative in explaining the etiology behind the observed variation.
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A glimpse at the current practice of blood transfusion in the pediatric emergency room, Medical City, Baghdad
p. 118
Hasanein Habeeb Ghali, Husam Mazar Azooz, Safa Abdulilah Faraj
DOI
:10.4103/ijh.ijh_28_20
BACKGROUND:
The onus of red blood cell transfusions in the pediatric emergency rooms (ERs) is paramount, with no specified guidelines, and almost all current policies are based on the obtainable adult facts.
OBJECTIVES:
The aims were to study the current practice and indications of blood transfusion in pediatric ER.
PATIENTS AND METHODS:
This is a cross-sectional study that included 50 pediatric patients who were admitted to the ER of Children Welfare Teaching Hospital, Medical City, Baghdad, from February to May 2017. The patients were admitted to the ER for different complaints and during their admission, they received a blood transfusion. The decision of blood transfusion was made by the most senior physician at different times. Patient data were tabulated and processed using Statistical Package for the Social Sciences (SPSS) for Windows.
RESULTS:
The age ranged from 15 days to 13 years, with a mean of 4.9 years. The main complaint was pallor in 26%, followed by bleeding (14%). Hemolysis due to presumptive glucose-6-phosphate dehydrogenase was the major diagnosis in 16 (32%) patients. The hemoglobin ranged from 2 to 11 g/dl, and the mean was 5.6 g/dl. The majority of decisions (28 cases, 56.0%) were made by the third-year resident, who is the second call on duties. Seven patients were below 4 months of age (14%), one of them received transfusion without logical indication. Forty-three (86%) patients were older than 4 months of age, of whom 13 (26%) patients received transfusion without logical scientific bases. The stay in ER ranged from 5 h to 5 days, and the mean was 26.5 h. The duration of transfusion was ranging from 60 min to about 6 h, with a mean of 3 h.
CONCLUSIONS:
The study showed major pitfalls in the management of patients with anemia. The main obstacles to implementation are the lack of trained staff.
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Soluble P- and E-selectin levels as determinants of vaso-occlusive crises among sickle cell anemia patients in a tertiary hospital Northwestern Nigeria
p. 123
Sani Awwalu, Abdulaziz Hassan, Aisha Indo Mamman, Abdul Wahab Alhassan, Ismaila Nda Ibrahim, Ibrahim Usman Kusfa, Aliyu Dahiru Waziri
DOI
:10.4103/ijh.ijh_29_20
BACKGROUND:
Vascular E- and P-selectins play important roles in sickle cell anemia (SCA), vaso-occlusive crises (VOC); however, the extent to which they determine VOC has not been studied.
OBJECTIVES:
The aims of the study were to assess vascular selectin levels and the extent to which they determine VOC among SCA patients.
SUBJECTS AND METHODS:
This was a cross-sectional comparative study conducted among patients with SCA in a tertiary hospital, Northwestern Nigeria. Eighty-eight participants were enrolled (44 each in VOC and steady state). Soluble E (sE) and P (sP) selectin levels were assessed using enzyme-linked immunosorbent assay technique. The frequency of blood transfusion and bone pains was collated.
RESULTS:
Patients with VOC compared to those in steady state had higher levels of sP-selectin (5.5 ± 4.6 ng/ml vs. 3.2 ± 0.8 ng/ml,
P
= 0.001) and mean rank sE-selectin (53.2 vs. 35.8,
P
= 0.001). The odds ratio (OR) for sE-selectin levels and VOC was 1.135 (
P
= 0.009), while that of sP-selectin was 2.693 (
P
= 0.002). The adjusted ORs for sE-selectin and sP-selectin were 1.184 (95% confidence interval [CI]: 1.021, 1.373) and 3.748 (95% CI: 1.475, 9.524), respectively.
CONCLUSIONS:
Patients in VOC have elevated sP-and E-selectin levels. sP-selectin level is a better predictor of VOC compared to sE-selectin.
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Hodgkin's lymphoma of the childhood: Experience of single hemato-oncology center
p. 127
Entisar Hadi Al-Shammary, Mohammed Jassim Al-Lami
DOI
:10.4103/ijh.ijh_30_20
BACKGROUND:
Hodgkin's lymphoma (HL) is a malignant lymphoma that accounts for approximately 7% of childhood cancers. The incidence of HL in childhood varies by age; it is the most common childhood cancer in the age group of 15–19 years.
OBJECTIVE:
The objective of the study is to determine the clinical presentation and outcome of HL among children under 15 years of age.
PATIENTS AND METHODS:
A retrospective study carried out at the hemato-oncology department of child's central teaching hospital included 46 HL patients under 15 years of age who were diagnosed over 6 years from January 2010 to December 2015. Demographic characteristics, histopathological and clinical features, treatment modalities, response to treatment, and outcome were obtained from the records files of the patients.
RESULTS:
Among a total of 46 children with HL, there were 33 (71.7%) males and 13 (28.3%) females; the male-to-female ratio was 2.1:1; the mean age of this study was 8.3 years. Lymphadenopathy was the most common sign, with cervical lymph node being the most common primary site of involvement. 47.8% of the atients present with Stage II, and the mixed cellularity histopathological subtypes were the common recognized subtype. Event-free survival (EFS) is 78.3%, and overall survival (OS) was 95.5%.
CONCLUSION:
Two-year OS (95.5%) and EFS (78.3%) of the HL patients were accepted as compared to other studies.
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Mutational analysis of T315I in patients with chronic myeloid leukemia who did not respond to second-generation tyrosine kinase inhibitors
p. 131
Riyam Qusay Ibrahim Al-Jadir, Jaffar Nouri Alalsaidissa
DOI
:10.4103/ijh.ijh_31_20
BACKGROUND:
Chronic myelogenous leukemia (CML) is a hematological stem cell disorder that associates with mutations in BCR-ABL. Although mutations identified in more than 30 different amino acids, the highest degree of resistance was associated with single-point mutation T315I of the ABL gene.
OBJECTIVES:
This study aims to identify the prevalence of T315I mutation among CML patients who lost their Major molecular response to second-generation tyrosine kinase inhibitor (TKI) at the time of the study and its relation to failure response.
SUBJECTS AND METHODS:
A prospective cross-sectional study included 50 adult patients diagnosed with CML. We have used the Sanger sequencing polymerase chain reaction based methods for detection of T315I mutation.
RESULTS:
Out of 50 patients, 28 were female (56%). Patients' age was ranged between 23 and 63 years with a mean age of 42.8 ± 11.46 years. There was no expression of T315I mutation in any of 50 CML patients, however, there was another mutation have been detected which is rs2070997 (response to drug) and was positive in 18 patients. There were 8 (16%) patients that have rs2070997 mutation have primary failure on first-generation TKIs, while the other 10 (20%) patients were those who started second-generation TKIs directly.
CONCLUSIONS:
T315I mutation was not common in patients with CML-chronic phase. Other (BCR-ABL dependent or independent) mechanism could be responsible of TKIs resistance.
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Interplaying of regulatory T-cells and related chemokines in immune thrombocytopenic purpura patients
p. 138
Zeyad A Shabeeb, Yusur F Faraj, Majed M Mahmood, Baan Abdulatif Mtashar
DOI
:10.4103/ijh.ijh_40_20
BACKGROUND:
Chronic immune thrombocytopenic purpura (ITP) is an immune-mediated bleeding disorder, in which platelets are opsonized by autoantibodies directed against platelet surface membrane glycoproteins, and prematurely cleared and destructed by Fc-receptors on the surface of macrophages in the reticuloendothelial system.
OBJECTIVES:
This work is designed to show the contribution of lymphocyte subsets and platelet destruction in adult chronic ITP and role of infection.
MATERIALS AND METHODS:
Transforming growth factor-β1 (TGF-β1) was measured using ELISA, and the frequency of regulatory T-cell (Treg) profile (CD4+CD25+CD127−) was investigated by FCM in blood samples of 50 Iraqi ITP patients (35 on-treatment ITP patients and 15 newly diagnosed) along with 20 age-matched healthy people that act as controls, as well as all patients were breath tested for detecting
Helicobacter pylori
using urea breath test. The study was carried out in the National Center of Hematology, Mustansiriyah University.
RESULTS:
The results showed that although there was a significant reduction in Treg number in ITP patients compared with the control individuals (
P
< 0.001), the effect of treatment has shown a restored count of Tregs in comparison to the newly diagnosed ones (
P
= 0.002), while the assessment of cytokine serum level revealed that TGF-β1 was significantly increased (
P
= 0.001) in the on-treatment group of patients (TGF-β1 = 3.24 ± 0.3 ng/μl) in comparison with the nontreated group of patients (TGF-β1 = 1.75 ± 0.2 ng/μl). However, it was still significantly (
P
< 0.001) less than their values in the apparently healthy individuals (TGF-β1 = 9.0 ± 0.2 ng/μ). Moreover, 25 out of 50 (50%) showed positive results for the presence of
H. pylori
.
CONCLUSION:
The present study revealed that Treg and its cytokines may play a fundamental role in the pathophysiology of adult chronic ITP since they contribute to the maintenance of peripheral immune tolerance. However, a causal link between
H. pylori
infection and ITP diseases is considerable.
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Automated versus manual method for reticulocyte count: A comparative study in rural central India
p. 145
Trupti Ramkrushna Gorte, Abhay Vilas Deshmukh, Nitin M Gangane
DOI
:10.4103/ijh.ijh_42_20
BACKGROUND:
Reticulocytes are immature red blood cells that contain remnants of ribonucleic acid. Various reticulocyte parameters can help in the proper diagnosis of different anemias. It can be measured by manual as well as by automated method.
OBJECTIVE:
The aim is to compare between the manual and automated methods of reticulocyte count (RC) in anemia.
MATERIALS AND METHODS:
It was a laboratory-based cross-sectional study in which the comparison of RC by manual and automated method along with various reticulocyte parameters was done in 300 patients with anemia and 300 control samples matched for age and sex.
RESULTS:
The study cases included 146 females and 154 males. No statistically significant difference was found between the automated and manual count among the male (
P
= 0.77) as well as female patients (
P
= 0.61). No statistically significant difference was found in mean RC among the infants (
P
= 0.71), children (
P
= 0.59), and adults (
P
= 0.66) between automated and manual count. The difference between mean manual and automated RC was statistically significant only in the case of males in the macrocytic anemia group (
P
= 0.092). Among reticulocyte indices, mean immature reticulocyte fraction (IRF), mean reticulocyte volume (MRV), and reticulocyte hemoglobin cellular content (RHCC) was found to be statistically significant among all types of anemia (
P
= 0.001, 00001 and 0.0001 respectively) while it was insignificant in case of mean corrected RC (
P
= 0.89). A significant positive correlation was found between manual and automated RC method by using Pearson's correlation coefficient (
r
= 0.985,
P
= 0.0001).
CONCLUSION:
There was no significant difference between the automated and manual methods for reticulocyte counting. However, the manual method may be preferred as it is cost-effective; yet, it is laborious, time-consuming, need efficient technique, not suitable for heavily loaded laboratories and may be suitable for under-resourced laboratories. However, the automated method is preferred as it is fast, highly precise, and it is mandatory for certain diseases where reticulocyte parameters are required as a statistically significant difference was found among the different parameters such as IRF, MRV, and RHCC.
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Evaluation of anti-A and anti-B IgM and anti-AB IgG antibody titers in Group O blood Iraqi donors
p. 150
Huda Hadi Jassim, Hind Shaker Al-Mamoori
DOI
:10.4103/ijh.ijh_43_20
BACKGROUND:
Blood group O is transfused as a universal blood group in emergencies. The red cells of blood Group O possess no major ABO antigens, while the blood Group O plasma contains naturally occurring IgM anti-A and anti-B and cross-reacting IgG anti-AB antibodies. Thus, the transfusion of blood Group O units may cause marked destruction of the recipients' A or B red cells manifested as severe acute hemolytic transfusion reactions owing to potent ABO hemolytic antibodies.
OBJECTIVES:
This study was done to estimate the titers of anti-ABO antibodies in Group O blood donors and to suggest a possible scheme to introduce as a routine testing for high ABO antibodies' titers in blood Group O donors in Iraqi blood transfusion centers.
SUBJECTS AND METHODS:
Two hundred blood Group O Iraqi healthy blood donors (192 males and 8 females, age range: 18–59) were chosen to evaluate the titer of ABO antibodies (IgM and IgG) in their serums using tube test.
RESULTS:
The prevalence of high-titer anti-A and anti-B IgM antibody at 1:50 dilution in this study was 98.5% and 97.5%, respectively (
P
< 0.05). The prevalence of high-titer anti-AB IgG measured by dithiothreitol and 2-mercaptoethanol method of at least 50 was 63% and 74%, respectively (
P
< 0.05).
CONCLUSIONS:
There is a high prevalence of high-titer anti-A and anti-B IgM and IgG antibodies among blood Group O Iraqi donors. The detection of IgG antibodies is more practical by using dithiothreitol method as it needs much shorter incubation time; however, 2-ME is more sensitive. Detection of high-titer anti-A IgM antibody in dangerous universal donors, by immediate spin at 1:50 dilution, can be used alone in practice, replacing the need to use all portfolio of tests of anti-ABO antibodies.
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Assessment of beta-2 microglobulin and CD49d in patients with chronic lymphocytic leukemia pre- and posttherapy
p. 155
Abdulameer Nasser Al-Rekabi, Alaa Fadhil Alwan, Naseer Khaleel Alobaidi
DOI
:10.4103/ijh.ijh_44_20
BACKGROUND:
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia among adults in Western countries; however, it is relatively rare in Asia. It is characterized by abnormal proliferation of lymphocytes in the blood, bone marrow, and lymphatic tissue. The measurement of serum beta-2 microglobulin (B2M) is essential for baseline workup of multiple myeloma and follicular lymphoma patients. CD49d, an adhesion molecule mediating cell-to-cell and cell-to-extracellular matrix interactions, represents a novel and the most reliable immunophenotypic marker regarding prognosis and independent of other markers.
OBJECTIVES:
This study aimed to assess the level of B2M and CD49d in serum CLL patients and correlates them with treatment response.
PATIENTS AND METHODS:
this is a prospective cohort study conducted on 70 patients with CLL and 40 healthy people as a control group. Patient groups were divided into two groups: The first group included 38 patients before receiving treatment and the second group included 32 patients posttreatment. Diagnosis was based on lymphocyte count of >5 × 109/L and immunophenotyping. The measurement of level B2M and CD49d in serum patients was done using enzyme-linked immunosorbent assay.
RESULTS:
there were 53 males and 17 females, the mean age was 59.12 ± 14.23, and the most clinical presentation was lymphadenopathy. Regarding the mean of B2M, it was 2.19 ± 0.86, 1.86 ± 0.58, and 1.41 ± 0.44 in the pre, post, and control groups, respectively, with
P
= 0.0001. Regarding the mean of CD49d, it was 0.22 ± 0.15, 0.30 ± 0.44, and 0.19 ± 0.13 in the pre, post, and control groups, respectively, with
P
= 0.211.
CONCLUSION:
this study showed that CD49d has no clinical impact on the treatment outcome, yet B2M has an important prognostic factor in deciding patients in advance stage.
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Study of hematological parameters in patients with coronavirus disease 2019 in Basra
p. 160
Qutaiba Muslim Dawood, Ziyad Tariq Al-Hashim, Basim A A Al Hijaj, Rawshan Zuhair Jaber, Asaad Abdulameer Khalaf
DOI
:10.4103/ijh.ijh_49_20
BACKGROUND:
Coronavirus disease 2019 (COVID-19) is a novel infectious disease that is caused by SARS-CoV-2, a newly emergent coronavirus, was first recognized in Wuhan, China, in December 2019. Genetic sequencing of the virus suggests that it is a betacoronavirus closely linked to the SARS virus. COVID-19 disease associated with hematological parameter changes like changes in complete blood picture and coagulopathy. Numerous observational studies have suggested that the neutrophil-to-lymphocyte ratio (NLR) and lymphocyte proportion and the platelet-to-lymphocyte ratio (PLR) are inflammatory markers.
OBJECTIVES:
The aim was to study the changes in hematological parameters in patients with COVID-19 in relation to the severity and outcome of the disease.
PATIENTS AND METHODS:
A prospective study had been done during the period of 3 months that extends from March to May 2020. From 543 patients admitted to the Basra Teaching Hospital COVID-19 wards, 112 medical reports of patients had been randomly selected. The demographic and blood test results for each patient were collected and followed the patient disease severity and the outcome. None of the selected patients had chronic disease or chronic use of medicine (s) that might affect the blood indices. Further analysis and statistics were done by SPSS software.
RESULTS:
The study showed that females were more than males 56.25% and 43.75%n respectively. Severity categorization showed that majority of cases were mild (88, 78.58%). Furthermore, the study reveals that 11 (9.82%) had leukocytosis, 6 (5.4%) of patients with leukopenia, 6 (5.36%) of patients with lymphopenia, and 16 (14.28%) of patients with neutrophilia. Seven patients (6.25%) had thrombocytosis while 6 patients (5.36%) presented with thrombocytopenia. Neutrophil/lymphocyte ratio in this study showed 28/112 (25%) patients with N/L ratio ≥3.1. Of those patients with high N/L, 39.29% were aged ≥65 years. All the patients with severe disease had high N/L (
P
= 0.000). The study showed that PLR ≥ 626 was found in 13.39% (15/112) of the studied patients, all of them had either severe (73.3%) or moderate (26.67%) disease which was highly significant when compared to low ratio (
P
=0.000).
CONCLUSION:
The study showed that the severity of COVID19 was associated with lymphopenia, monocytosis, and elevated NLR and PLR values. On the other hand, both values (NLR and PLR) could be used as hematological predictors for disease severity and the outcome of patients with COVID-19.
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CASE REPORT
Imported vivax malaria: A case report and a literature review
p. 166
Basma Dawood Hanoon
DOI
:10.4103/ijh.ijh_8_20
Imported malaria is defined as an infection acquired in a malaria-endemic area but diagnosed in a nonendemic country after the development of clinical symptoms. The fatality rate of malaria in the nonendemic area was 60 times higher than that in endemic areas, mainly because of the late diagnosis and treatment. Here, we report a 23-year-old Bangladesh male worker in a cleaning company was admitted to the Al Nuaman Teaching Hospital on May 22, 2018, with high-grade fever and abdominal pain of 2 days duration. In medical history, he had been diagnosed with malaria since childhood. The case report is presented with a review of the literature. Microscopy remains the gold standard diagnostic tool of malaria, thick and thin smears ± rapid diagnostic tests should be performed on all febrile returned travelers from risk areas.
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LETTER TO EDITOR
Assessment of anthracycline-induced long-term cardiotoxicity in patients with hematological malignancies
p. 170
Ala Ali
DOI
:10.4103/ijh.ijh_13_20
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