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   Table of Contents - Current issue
July-December 2022
Volume 11 | Issue 2
Page Nos. 91-200

Online since Wednesday, December 14, 2022

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AryoSeven RT (Coagulation factor VIIa, recombinant) safety and efficacy study among congenial factor VII deficient patients in Iraq p. 91
Nidal Karim Al-Rahal, Ahmed Shemran Alwataify, Safa Mohammed Shawkat, Israa Almusawi, Azeezah Mohammed Mohsin
BACKGROUND: Recombinant activated factor VII (FVII) is a product onetime evolved to stop bleeding occurring in hemophilia A and B patients with inhibitor, congenital FVII deficiency, Glanzmann disease, and in life-threatening bleeding. AIM: The aim was to evaluate the safety and efficacy of the coagulation factor VIIa, recombinant (AryoSeven RT) among congenital FVII deficiency patients at different centers in Iraq. METHODOLOGY: This is a prospective, observational, noninterventional study done at 5 medical centers in Iraq and it included 22 patients with FVII deficiency (congenital form) older than 14 years of age. Patients are recorded and followed for 6 months and they are subjected to AryoSeven RT depending on each patient individually. There were 3 main visits and 3 unscheduled visits for each patient during the study. Effectiveness evaluation was performed 6 h after each intervention. Adverse drug reactions related to the administration of AryoSeven RT were reported for each patient during each visit. RESULTS: A total of 22 participants were enrolled, classified into 18 (82%) were female and 4 (18%) were male. The mean age was 27.5 ± 14.0 years. Among 91 bleeding events, AryoSeven RT efficacy was effective in 89 events, excellent in 1 event, and partially effective in also 1 event. There was a reduction of PT from baseline (57.3 ± 15.2 s) to (13.9 ± 6.2 s) after 1st dose of AryoSeven RT and more reduction after 2nd dose of therapy (13.4 ± 4.4 s) and these were statistically significant (P = 0.001). Regarding FVII activity, there was a significant increase from baseline (8.4% ± 8.0%) to (95.8% ± 46.6%) after 1st dose and (131.8% ± 40.1%) after 2nd dose of AryoSeven RT with P = 0.001 for both. No major adverse events were reported except for headache in one participant (4.5%), and injection site reactions in three participants (13.6%).) CONCLUSION: AryoSeven RT is safe and effective clinically and by laboratory data in stopping bleeding in patients older than 14 years with inherited FVII deficiency.
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Clinical profile of sickle cell disease patients attending pediatric department of a tertiary care hospital and Sickle-Thal center: A cross-sectional study p. 98
Naresh B Tayade, Sanket S Pande, Manjusha K Deotale
BACKGROUND: Sickle cell disease (SCD) is a major public health problem in certain states of India associated with morbidity and mortality in children. The characteristics and clinical features of SCD are different in India as compared to those in developed countries with considerable clinical diversity. AIMS: This study aimed to describe clinical features in patients with SCD at the time of diagnosis and first visit. SETTING AND DESIGN: Department of Pediatrics at tertiary care hospital or Sickle-Thal Society Amravati,India (Both Collectively called as Unit). Design: Cross sectional Study. MATERIALS AND METHODS: All SCD patients 0–18 years, attending unit from October 1, 2019, to March 31, 2021, were included in the study. Data collected were based on history, clinical examination, and review of old records. The sample size was 153. STATISTICAL ANALYSIS: Data was analyzed by using software statistical packages in social sciences (SPSS) version 16. Mean, standard deviation (SD), proportions were calculated and Chi -square test of significance was used. RESULTS: Of 157 SCD patients (145 families), 90 (57.32%) were males and 67 (42.68%) females. The mean age of diagnosis and unit visit was 53.59 and 115.74 months, respectively. At diagnosis, paleness (68%), pain (54%), and fever (45%) were common complaints with other nonspecific complaints. Pallor (97.45%) and palpable spleen (53.90%) were common signs at the first unit visit. The spleen was palpable in 65 (42.48%) even after 5 years. CONCLUSION: SCD patients had paleness, pain, and fever as the most common complaints. Palpable spleen was seen even after 5 years of age. Early suspicion is key for diagnosis.
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Microalbuminuria among children and adolescents with sickle cell disease p. 103
Meaad Kadhum Hassan, Lamia Mustafa Al-Naama, Sammer Muayed Jawad
BACKGROUND: Sickle cell nephropathy, a heterogeneous group of renal abnormalities resulting from complex interactions of sickle cell disease (SCD)-related factors and non-SCD phenotype characteristics, is associated with an increased risk for morbidity and mortality. AIMS: The aims of this study were to determine the frequency of microalbuminuria (MA) among pediatric patients with SCD and to determine risk factors for MA among those patients. SUBJECTS AND METHODS: A case–control study was carried out on 120 patients with SCD, 2–18 years old, registered at Basrah Center for Hereditary Blood Diseases, and 132 age- and sex-matched healthy children were included as a control group. Investigations included complete blood panel, blood urea, serum creatinine (Cr), urinalysis, and urinary albumin-to-Cr ratio (ACR). Logistic regression analysis was used to assess the predictors of MA. RESULTS: Among SCD patients, 39 (32.5%) had MA compared to 6 (4.5%) in the control group. The mean levels of blood urea, serum Cr, and ACR were significantly higher, and the urine-specific gravity was significantly lower in SCD patients than in the control group (P < 0.05). Logistic regression analysis revealed that frequent painful crisis (odds ratio [OR]: 12.146, confidence interval [CI]: 3.439–42.952), high serum ferritin (OR: 8.146, CI: 1.802–36.827), deferoxamine therapy (OR: 23.423, CI: 3.961–60.509), and female sex (OR: 4.590, CI: 1.225–17.202) are independent risk factors for MA (P < 0.05). CONCLUSION: The frequency of MA was high among our pediatric SCD patients. Risk factors for MA include female sex, nutritional factors, painful episodes, and iron overload. This is important for planning for future follow-up and management of this common disease in our locality.
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The frequency of HLA A, B, C, DP, DQ, DR allele in patients of Turkish and Syrian nationals with allogeneic stem cell transplantation p. 110
Ahmet Kaya, Mehmet Ali Erkurt, İrfan Kuku, Emin Kaya, İlhami Berber, Soykan Biçim, Emine Hidayet, Fatma Hilal Yağin, Ahmet Sarıcı
BACKGROUND: In allogeneic stem cell transplantation, donor and recipient HLA tissue compatibility is essential for the success of stem cell transplantation. HLA tissue compatibility percentage is the most important parameter that increases the success of transplantation in donor selection. Pretransplant HLA tissue typing can be looked at in low and high resolution according to the facilities of the center and the urgency of transplantation. Many centers evaluate HLA HLA-A, B, C, DP, DQ, DR tissue types before allogeneic bone marrow transplantation. HLA tissue types differ in many races and even between unrelated individuals of the same race. AIMS: This study aimed to show the common human leukocyte antigen (HLA) rates and differences in Syrian and Turkish ethnicity patients who underwent allogeneic stem cell transplantation in our center. MATERIALS AND METHODS: HLA tissue similarities between Turkish and Syrian patients were revealed by examining the HLA tissue records of Turkish and Syrian patients who applied to the bone marrow transplant unit of Inonu University Turgut Ozal Medical Center between December 2009 and November 2021 for allogeneic stem cell transplantation. RESULTS: As a result of our study, it has been observed that there are similarities in terms of HLA A*02, HLA B*35, HLA C 04,07,12, HLA DP*02,04,11 HLA DQ*02,03,05,06, HLA DR*01,03,11,13 in Turkish and Syrian patients. High resolution HLA subgroups of the patients are shown in Tables three and four. CONCLUSION: In allogeneic stem cell transplantation, there may be similar HLA tissue types among ethnic groups.
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A role of interim positron emission tomography/computed tomography scan in the management of Hodgkin lymphoma: A single-center study in a developing country p. 118
Saya Azeez, Ahmed Khudair Yassin, Nawsherwan S Mohammad, Kawa M Hassan, Zeki A Mohamed, Ranan Kardagh Polus, Hawar Gh. Khudhur
BACKGROUND: The Hodgkin lymphoma (HL) showed a good prognosis in Erbil city. However, a number of deaths were reported. The monitoring and treatment strategies of HL had improved in the last decade. AIMS: The aims of this study were to evaluate the prognostic value of interim positron emission tomography/computed tomography (PET/CT) scan in HL patients and predicting the survival outcome in addition to effect on progression-free survival (PFS) of HL patients. PATIENTS AND METHODS: A retrospective cross-sectional study was conducted in Nanakali Hospital in Erbil city, Kurdistan region, Iraq, through reviewing data of patients with HL from January 1, 2014, to December 31, 2019, on a sample of 75 HL patients. The diagnosis of HL was accomplished by the physician regarding lymph node biopsy by the World Health Organization criteria. The PET/CT scan was done after two treatment cycles. PET/CT of HL patients was done in a private center in Erbil city (Media Diagnostic Center). RESULTS: The mean overall survival duration of HL patients was 5.39 years with an overall survival rate of 92%, and the mean PFS duration of HL patients was 5.04 years with PFS rate of 76%. The mean overall survival of HL patients with positive interim PET/CT (4 years and rate 75%) was lower than the mean overall survival of HL patients with negative interim PET/CT (7.2 years and rate of 95.2%) with a significant difference (P < 0.001). CONCLUSIONS: The interim PET/CT scan is useful in prognosis of patients with HL and predicting overall survival and mortality.
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Analysis of single-donor plateletpheresis procedure parameters and its association with yield in a blood center of Eastern India p. 125
Girija Nandini Kanungo, Suman Sudha Routray, Milind Agrawal, Aruna Sahu, Debasish Mishra
BACKGROUND: Single-donor platelets are most commonly used to prevent and treat bleeding in thrombocytopenic patients. Plateletpheresis machines should take less procedure time, optimal yield, and donor comfort. This study aimed to compare different donor parameters such as predonation platelet count and hematocrit (Hct), blood volume (BV) processed, and blood group association with yield. MATERIALS AND METHODS: A retrospective study was conducted in the transfusion medicine department and blood center for 12 months. One hundred and fifty-nine plateletpheresis procedural data on Trima Accel were collected from the register and compiled in the Excel sheet. Donor procedure variables were calculated as mean ± standard deviation. Correlation between donor parameters with yield and anticoagulant (AC) infused to the donor with run time was analyzed using Pearson correlation coefficient in Excel sheet with Microsoft Windows 7 software. The association between blood group and yield was analyzed in GraphPad 9 software, CA. P < 0.05, <0.001 was statistically significant. RESULTS: Predonation platelet count (r = 0.61, P < 0.001) and BV processed (r = 0.18, P < 0.05) were positively correlated with yield. Predonation hemoglobin, Hct, and Run time was positively correlated with AC infused to the donor (r = 0.72, P < 0.001). Different blood groups had no statistical significance association with yield. CONCLUSION: In our study, predonation platelet count directly relates to yield; hence, donors with high platelet count may be considered for better yield. Run time also had a direct relation with AC going to the donor; minimal run time also had a role in preventing adverse effects on the donor.
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Face masks and polycythemia is the standard hemoglobin cutoff valid in the pandemic? p. 130
Samet Yaman, Semih Basci, Gökhan Turan, Bahar Uncu Ulu, Tugçe Nur Yigenoglu, Taha Bahsi, Haktan Bagis Erdem, Mehmet Sinan Dal, Merih Kizil Çakar, Fevzi Altuntas
BACKGROUND: Polycythemia is a common reason for patients' admissions. With the introduction of COVID-19, face masks reached very common usage in the population. Masks may cause some degree of hypoxia that may result in high hemoglobin in healthy individuals. Here, we aimed to investigate the frequency of patients applying for high hemoglobin and tested for possible polycythemia vera (PV) in the pandemic era. MATERIALS AND METHODS: We collected patients who applied to the hematology outpatient clinic between March 2019 and April 2021 for the study. The research was carried out at a single center at Ankara Oncology Training and Research Hospital. We collected demographic data such as age and sex, laboratory parameters such as complete blood count and erythropoietin level, concomitant diseases, smoking history, and spleen size. RESULTS: The median age of the cohort was 41 (16–83). Groups were different regarding age (P = 0.04). Groups were similar regarding gender (P = 0.350). Comorbidities were similar in both groups. Smoking was more frequent in the pre-COVID era group (P = 0.046). The frequency of the Janus kinase 2 (JAK2) test order was 102 examinations out of 7920 for the pre-COVID era and 152 examinations out of 6087 for the COVID era; this was statistically significant (P < 0.001). CONCLUSION: Clinicians may need to re-evaluate the threshold of hemoglobin levels to order JAK2 tests in the pandemic era, and the significance of mildly elevated hemoglobin may be neglected while testing for potential PV.
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Clinical significance of serum sCD23 and B-cell maturation antigen levels in patients with chronic lymphocytic leukemia p. 134
Talib Mohammed Ameen, Haithem Ahmed Al-Rubaie
BACKGROUND: Chronic lymphocytic leukemia (CLL) is a malignancy of mature appearing clonal B lymphocytes where there is a progressive accumulation of leukemic cells in peripheral blood, bone marrow, and secondary lymphoid tissues as a consequence of defective apoptosis and survival signals derived from the microenvironment. The soluble CD23 (sCD23) is a 25 kDa fragment that can be found in serum, plasma, and urine in patients with CLL. It is a B-cell growth factor. B-cell maturation antigen (BCMA) is a member of the tumor necrosis factor superfamily, it enhances the survival and proliferation of mature B cells and plasma cells through signal transduction of the B-cell activating factor and a proliferation-inducing ligand. AIMS: The aims of this study were to assess the serum levels of sCD23 and BCMA in newly diagnosed CLL patients and to correlate them with clinical Binet staging and other hematological and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 54 newly diagnosed CLL patients and 27 healthy controls. Diagnosis of CLL patients was based on lymphocyte count of >5 × 109/L and immunophenotyping. The serum levels of sCD23 and BCMA were measured in both groups using an enzyme-linked immunosorbent assay. RESULTS: Serum levels of sCD23 and BCMA were significantly higher in CLL patients in comparison with control group (P < 0.001 for both). There was a significant direct association between serum levels of sCD23 and BCMA with the clinical Binet stage of the disease (P < 0.001 for both). sCD23 showed significant correlation with hemoglobin (Hb) level (P < 0.001), total white blood cell (WBC) count (P = 0.001), lymphocyte count (P < 0.001), platelet count (P = 0.017), B-symptoms (P = 0.001), and splenomegaly (P = 0.019), whereas BCMA has significant correlations with Hb level, total WBC count, lymphocyte count (P < 0.001 for each one), B-symptoms (P < 0.001), lymphadenopathy (P = 0.001), splenomegaly (P = 0.024), and hepatomegaly (P = 0.04). CONCLUSIONS: The levels of serum sCD23 and serum BCMA increase with advancing Binet stages of the disease indicating their possible usefulness as good and reliable parameters for prognostic evaluation in CLL patients. The significant correlation of serum sCD23 and serum BCMA with hematological parameters and clinical features render them as reliable tumor burden markers in CLL patients.
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Coagulopathy in hospitalized COVID-19 patients: A single-center experience p. 139
Marwa Kasim Ahmed, Ali Muhammed Jawad Almothaffar
BACKGROUND: The coronavirus disease pandemic had spread across all countries. SARS-COV-2 infected up to date millions of people and the threat remains there for others. A lot of SARS-19-infected people with critically ill symptoms admitted to intensive care facilities had developed respiratory failure, coagulopathy, and organ failure. AIMS: The aims of this study were to investigate the prevalence and risk factors associated with coagulopathy in COVID-19 patients who were admitted to the Private Nursing Home Hospital in Baghdad/Iraq. PATIENTS AND METHODS: A case series study was conducted in the Nursing Home Hospital in Baghdad, Iraq, from October 2020 to December 2021. A total of 150 cases were included in this study with confirmed COVID-19 infection by polymerase chain reaction of throat or nose swab. These patients were admitted to two isolation wards (isolation intensive care unit for critical cases and medical ward isolation unit for moderately severe cases). Baseline and follow-up characteristics and laboratory parameters of coagulopathy (blood counts, prothrombin time [PT], partial thromboplastin time, D-dimers, and plasma fibrinogen) were obtained for each patient. The ISARIC 4C has been used for risk stratification (4C Mortality Score is a prognostic model for clinical deterioration among hospitalized adults with community-acquired or hospital-acquired COVID-19, it is used for stratifying and predicting mortality in COVID-19 patients on arrival in hospital). RESULTS: The mean age across patients was 56.6 ± 15.7 years (range: 14–90 years). Males were representing the majority of cases (63.3%) with a male-to-female ratio of 2:1. The mean 4C score of patients was 10.3 ± 4.9 (range: 0–20 points). The risk group stratification showed that many patients had high risk (42.7%), and only 10.7% of patients had low scores. There were 86 (57.3%) patients who developed coagulopathy during the follow-up period and 46.7% of total patients died. There was a significant association between developing coagulopathy with higher risk group and death in COVID-19 patients (P < 0.05), while age and gender did not demonstrate a significant association. Furthermore, there was a significant association between respiratory failure, patients with cancer, patients with stroke, higher computed tomography lung involvement, lower SPO2, the presence of shock, and pulmonary embolism with the development of coagulopathy (P < 0.05). There were significant higher baseline levels of the neutrophil count, PT, D-dimer, and ferritin among patients who developed coagulopathy, while there were significant lower baseline levels of platelet count and serum albumin among patients who developed coagulopathy. CONCLUSIONS: The development of coagulopathy in the course of severe SARS-COV-2 infection is associated with different severity biomarkers and is associated with excessive mortality.
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Assessing the impetus and impediments to donate coronavirus disease-2019 convalescent plasma: A survey study from a tertiary care Indian blood center p. 145
Manish Raturi, Yashaswi Dhiman, Mansi Kala, Naveen Bansal, Anuradha Kusum
BACKGROUND: Amidst the raging coronavirus disease-2019 (COVID-19 pandemic), COVID-19 convalescent plasma (CCP) therapy emerged as an important experimental therapy. The majority of the research studies have focused on determining the safety and efficacy of CCP in the management of COVID-19 and little attention has been paid to the source of CCP, i.e., the CCP donor recruitment. The main challenge at our blood center was maintaining high spirits and continuous motivation among the volunteers to keep donating CCP. With this background, our primary aim was to observe the impetus and impediments of potential CCP donors among the SARS-CoV-2 recovered individuals. MATERIALS AND METHODS: All the potential CCP donors who met the inclusion criteria were telerecruited, i.e., contacted telephonically by trained personnel of the blood center. Donors were informed about CCP and its harvest procedure. Subsequently, donors were presented with 10 statements to assess the potential impetus (motivators) and impediments (barriers) toward donating CCP. In addition, SARS-CoV-2 IgG antibody titers were performed using chemiluminescence assay in donors who passed the predonation screening. RESULTS: Based on their medical records, a total of 96 potential CCP donors were contacted telephonically for inclusion in the study. Among these 68.75% (n = 66/96) individuals expressed their willingness to donate CCP. “Altruism from adversity” was the most common motivational factor among voluntary donors (47.8%), whereas kinship was the strongest motivational factor among replacement donors (40%). Logistical reasons such as their distance from the blood center (33.3%) were the most common reason for not donating CCP. About 21.2% (n = 18/66) of the donors were deferred due to absent or low levels of anti-SARS-CoV-2 IgG antibodies. CONCLUSIONS: Motivation factors such as “altruism from adversity” are more common in CCP donation. At the same time, most of the impediments to CCP donation were similar to those of the routine apheresis procedure. Knowledge of these motivators and barriers should form the cornerstone of plasma therapy-based donation program in any future pandemic.
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Outcome of adult acute lymphoblastic leukemia, a single-center experience p. 151
Hawar Ghareb Khudhur, Kawa Muhamadamin Hasan, Ranan Kardagh Polus, Ahmed Khudair Yassin, Nawsherwan Sadiq Mohammad, Saya Salahaldeen Najmaldeen
BACKGROUND: Adult acute lymphoblastic leukemia (ALL) has a dismal outcome within the optimal situations as the 5-year overall survival (OS) is not reaching 40%. OBJECTIVE: The objective of this study was to evaluate the outcome of adult ALL in Erbil, Iraq, and to see the factors predicting better survival among them. PATIENTS AND METHODS: We analyzed data from 71 adult ALL patients aged 14–81 years (mean age: 28.6 years). Diagnosis is based on the peripheral blood film, bone marrow examination, and flow cytometry. RESULTS: The median OS was 21 months and the median relapse-free survival (RFS) was 13 months. The 3-year OS and the 3-year RFS were 49.3% and 40.8%, respectively, while the 5-year OS and the 5-year RFS were 37.5% and 32.5%, respectively. CONCLUSION: Factors predicting better survival included in the study; age at diagnosis, remission achievement at first induction, risk assessment, and allogeneic stem cell transplantation optimal response.
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Clinico-hematological features and outcome of patients affected by Congo–Crimean hemorrhagic fever: An experience from a single center p. 158
Mohammad Usman Shaikh, Natasha Ali, Ayesha Majeed Memon
BACKGROUND: Congo–Crimean hemorrhagic fever is a tick-borne zoonotic viral disease caused by Crimean–Congo hemorrhagic fever virus (CCHFV). The outbreak in Pakistan is increased during Eid-ul-Adha. We describe a cluster of cases that presented to our hospital. AIMS: The aim of this study was to determine the outcome of Crimean–Congo hemorrhagic fever-positive cases from January 2011 to August 2019. MATERIALS AND METHODS: Retrospective data were retrieved using the International Classification of Diseases version 9. We received 2101 samples for testing. Polymerase chain reaction (PCR)-positive cases were included in the study. History of bleeding and animal contact was recorded. Variables analyzed were age, gender, profession, and coinfection with other viral illnesses. RESULTS: A total of 70 PCR-positive cases were included in the study (frequency 3.3%). Sixty-one were males and nine were females. Fever was present in all cases. Epistaxis was noted in 54% of patients. Thrombocytopenia was present in all patients. Eighteen patients were butchers, six were shopkeepers, nine students, and few laborers. All females were housewives. Twenty-two patients had a history of contact with animals. Seven patients were coinfected with viral hepatitis. There were 23 (33%) deaths. CONCLUSION: Overall mortality was 33%. Twenty-two patients had a history of contact with animals. There is a strong need for public education, especially during the month of Eid-ul-Adha.
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Increased thrombomodulin level in hypertensive disorders of pregnancy p. 163
Zainab Abdul Abbas Hashim, Bassam Mohamed Hameed
BACKGROUND: Endothelial dysfunction is a likely pathogenic mechanism in hypertensive disorders of pregnancy leading to a hypercoagulable state. OBJECTIVE: The present study aims to measure thrombomodulin (TM) in patients with preeclampsia (PE) and gestational hypertension (GH) and compare them with healthy pregnant control and its relation to disease severity and associated hematological parameters. MATERIALS AND METHODS: This cross-sectional study was done for 80 participants, 30 preeclamptic, 30 GH patients, and 20 healthy age-matched pregnant from all TM assays were done in by an enzyme-linked immunosorbent assay. Other hematological parameters including complete blood count, prothrombin time, and activated partial thromboplastin time where assessed in these patients. RESULTS: TM level was significantly higher in patients with PE when compared to both women with GH and normal pregnant women (P = 0.009) and (P < 0.001), respectively. Likewise, TM level was significantly higher in patients with GH when compared to healthy pregnant controls (P = 0.034). Plasma TM level was found to be 77% sensitive and 75% specific for the diagnosis of PE (the area under the curve was 0.835) at a 95% confidence interval. CONCLUSION: TM is significantly elevated in pregnant women with PE and GH and is associated with the severity of the disease.
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Frequency and types of transfusion reactions in pediatric population: A report from a tertiary care center in Pakistan p. 170
Jyoti Mohan Lal, Muhammad Hasan, Natasha Ali
Background: A transfusion reaction is an untoward reaction following blood transfusion. It can be immediate or delayed and further sub-classified into immune and nonimmune. The role of physicians and paramedical staff is important in recognition of reaction and notifying transfusion services by sending relevant material for workup. Objective: The aim of the current study is to see frequency and types of transfusion reactions in pediatric population in a tertiary care center. We also assessed the compliance of clinical staff to send the proper transfusion reaction workup in required time. Materials And Methods: This retrospective cross-sectional study was conducted in the section of hematology and transfusion medicine of a tertiary care hospital in Karachi, from January 2020 to December 2021 after the approval from Ethical Review Committee. The data were analyzed using SPSS version 20. Results: Of the 21,230 units dispensed and transfused, 36 (0.17%) transfusion reactions were noted. Allergic was the most frequent type 21 (58.3%). Red cells accounted for 28 (77.8%) of the reactions. In all cases, reaction forms were completely filled. Blood bags, posttransfusion ethylenediaminetetraacetic acid samples and urine samples in only 8 (22.2%) cases were received at blood bank within 2 h of reaction. Conclusion: Incidence of transfusion reactions was 1 in 590 units transfused. Allergic reactions were most common. No acute hemolytic or septic reaction noted. Practices regarding submission of transfusion reaction form along with required workup to the blood bank need improvement.
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Relation between FMS-like tyrosine kinase 3 factor and hematological parameter in acute lymphoblastic leukemia patients by flow cytometry p. 175
Zainab Samir Al-Ali, Bassam Mohammed
BACKGROUND: Acute lymphoblastic leukemia (ALL) is a heterogeneous disorder that is caused by the clonal expansion of immature lymphoid cells with a high rate among children more than adults. FMS-like tyrosine kinase 3 (FLT3) is a cellular receptor belongs to the Class III receptor tyrosine kinase family. The main expression of FLT3 on bone marrow (BM) cells especially CD34+ hematopoietic stem cells, early progenitor cells, dendritic progenitor cells, and other cells of organs (brain, placenta, and testis). Activation of FLT3 results in increased cell proliferation, decreased cell apoptosis, and inhibition of differentiation of cells. This study aims to detect the expression of the FLT3 cluster of differentiation antigen 135 (CD135) in childhood B-ALL patients. Moreover, to correlate this expression with hematological parameters include a complete blood count and BM examination findings and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 30 newly diagnosed pediatric ALL patients. Diagnosis of the disease was based on the blood film, BM examination findings, cytochemistry, and flowcytometry of peripheral blood (PB) and/or BM sample, 1 ml of PB and/or BM sample was collected in EDTA tubes for flowcytometry for detection of CD135. RESULTS: This study found that male patients were more than females with a male-to-female ratio (1.14:1) and a median age of 5 years. Most of the patients had a positive expression of the FLT3 receptor and according to NCI risk groups, 60% of patients fall in the standard risk and 40% in the high-risk group. There was a significant correlation between the level of FLT3 (CD135) and age but no significant correlation with hemoglobin, white blood count, platelets, and peripheral or BM blast percentage. CONCLUSION: In this study, the patients with positive FLT3 blast cells (which is a bad prognostic factor) were associated with good prognostic factors. This proves that FLT3 is an independent prognostic factor.
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Addison's disease in a lady with hemoglobin H disease p. 182
Nawshirwan Gafoor Rashid
Hemoglobin H (HbH) disease is alpha (α)-thalassemia characterized by the inactivation of three of four α-globin genes due to deletions with or without nondeletional α-thalassemia. HbH disease is not necessarily a benign disorder as has been generally thought. Here, we report a 33-year-old female who has a lifelong history of anemia without blood transfusions. but when she got married, during pregnancies developed symptomatic moderate-to-severe anemia necessitating infrequent blood transfusions. Later due to symptomatic anemias and increasing the frequency of blood transfusions, she underwent splenectomy, 3 years from splenectomy she developed a gradual dark skin discoloration with frequent hypotension and was diagnosed with Addison's disease which is a rare endocrine complication of thalassemia-induced iron overload.
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Central nervous system relapse in multiple myeloma: An unusual complication p. 186
Arun Vasudevan, Geetha Narayanan, Nileena Nayak
Central nervous system involvement in multiple myeloma (MM) is rare and carries a very poor prognosis. We report the case of a 62-year-old man diagnosed with MM who received induction chemotherapy with bortezomib, cyclophosphamide, and dexamethasone and achieved very good partial response. He developed seizures and altered sensorium while on maintenance chemotherapy. Magnetic resonance imaging of the brain showed leptomeningeal enhancement, and cerebrospinal fluid cytology was positive for plasma cells. His general condition worsened, and he died before starting any specific treatment.
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Pernicious anemia with unusual associations p. 189
CA Mansoor
Pernicious anemia (PA) is an autoimmune disease due to vitamin B12 deficiency secondary to Intrinsic Factor deficiency. A 65-year-old man presented with left lower limb swelling and pain for one week. He had loss of appetite and fatigue for one month. Investigations showed lower limb deep vein thrombosis and bicytopenia. On further evaluation, he was found to have PA, duodenal carcinoids, and primary biliary cirrhosis. He was managed with heparin (followed by warfarin), parenteral Vitamin B12, and ursodeoxycholic acid. He was referred to the gastroenterology department for management of duodenal carcinoid. On follow-up his blood counts were normal. We present a patient with PA who had three unusual associations simultaneously at the time of presentation which was never reported in the literature previously, to the best of our knowledge.
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Cross-allergic reactions between etoposide and penicillin in autologous bone marrow transplant patient p. 192
Alaa Hussein Alsajri, Mazin Abbas Shubber, Walid Al-Qerem
Etoposide is a chemotherapeutic agent that belongs to the podophyllotoxin drug class. Etoposide is used in treating many types of cancers including blood cancers. However, hypersensitivity reactions to etoposide and other chemotherapeutic agents are common. A 29-year-old female was admitted to the bone marrow transplant center for autologous hematopoietic stem cell transplantation. She was previously diagnosed with Hodgkin's lymphoma. The Lomustine, Etoposide, Cytarabine and Melphalan, (LEAM) protocol has been prescribed as a conditioning regimen before stem cell transplantation for this patient. On the 4th day of LEAM protocol, after the last day of the etoposide dose, the patient develops a severe allergic reaction to etoposide. After investigation, we found that the patient was allergic to penicillin which also appeared when the patient takes piperacillin + tazobactam. The possibility of cross-allergic reactions between etoposide and penicillin is unknown. The cross-allergic reactions between etoposide and penicillin are not reported in previous studies.
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Hemolysis and COVID-19 infection: A fatal combination – An interesting series of three cases p. 196
K. V. Vinu Balraam, Vikas Raj, Gurpreet Kaur Sagoo, Nidhi Garg, Nitin Sagar Taneja, Alok D Sen
COVID-19 has wreaked havoc ever since its inception and with the protean manifestations of the disease, it is imperative that progressively data are added to the literature. COVID-19 infection is a multisystem disorder with a wide range of clinical symptomatology. Recent information garnered has laid emphasis on pathological changes at microvascular level causing thrombotic/hemostatic defects, leading to the assorted clinical presentation. We present a consortium of three confirmed COVID-19 cases whose hospital course got convoluted with grave hematological complications in the form of hemolytic uremic syndrome and autoimmune hemolytic anemia. Regrettably, all three patients succumbed to their illness.
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