Journal of Haematology and Stem Cell Research <p><em>Journal of Haematology and Stem Cell Research (JHSCR)</em> is an official journal of Pakistan Society of Haematology, recognized by <strong>Higher Education commission of Pakistan in Y category)</strong>. It is a double blind double peer-reviewed being published biannual journal that delivers the premier quality peer-reviewed open access original research articles, reviews, and scholarly comment on pioneering efforts and innovative studies in the medical treatment of blood disorders. The journal has a strong clinical and pharmacological focus and is aimed at an international audience of clinicians and researchers in hematology and related disciplines, providing an online forum for rapid dissemination of recent research and perspectives in this area.</p> Pakistan Society of Haematology en-US Journal of Haematology and Stem Cell Research 2790-2277 <p><a href="" rel="license"><img src="" alt="Creative Commons License" /></a><br />This work is licensed under a <a href="" rel="license">Creative Commons Attribution-NonCommercial 4.0 International License</a>.<br />The licensor permits others to copy, distribute, display, and perform the work, as well as make and distribute derivative works based on it. The licensor permits others to copy, distribute, display, and perform the work for non-commercial purposes only.</p> Haematology Warriors – Played Well Nadeem Ikram Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-13 2023-07-13 3 1 1 2 Hepatitis E Virus Outbreak in an Internally Displaced Population of Afghanistan: Is it a Risk to Transfusion Safety? <p><strong>Objective:</strong> The objective of this study was to investigate the Hepatitis E virus (HEV) outbreak in an internally displaced population (IDP) of Afghanistan, specifically in the Jalalabad region near the Torkham border.</p> <p><strong>Methodology:</strong> Blood samples were collected from 544 suspected cases in IDP camps in Jalalabad, Afghanistan, near the Torkham border. The samples were screened for HEV antibodies (IgM and IgG) and alanine aminotransferase (ALT). Subsequently, the samples were transported to the Peshawar Regional Blood Centre in Pakistan for serological and molecular analyses. The data were stored in Microsoft Excel 2013 and analyzed by the IBM Statistical Package for Social Sciences version 24.0 Armonk, NY: IBM Corp. for frequency, percentage, mean, and standard deviation. The Chi-square test was used where appropriate, and a p-value of &lt; 0.05 was considered significant with a 95% confidence level.</p> <p><strong>Results:</strong> Out of the 544 samples serologically screened, 135 (24.81%) tested positive for HEV IgM, while 59 (10.84%) were reactive for HEV IgG. Among these samples, only 41 that were HEV IgM reactive also showed reactivity for HEV IgG. Within the cohort of 135 HEV IgM positive patients, 80 (59.25%) had donated blood for their family or friends at least once in the past three years, and all of them were males. Additionally, among the 135 HEV IgM positive samples, 86 (63.70%) tested positive for HEV RNA. The positivity frequency of HEV RNA was 100% (76/76) for symptomatic cases and 16.94% (10/59) for asymptomatic cases.</p> <p><strong>Conclusion:</strong> Our findings suggest that drinking river water was a potential source of HEV infection in this outbreak. It is prudent to consider hepatitis E as a potential risk to blood safety, especially considering that the majority of the infected cases were males with a history of recent blood donation. Furthermore, it is worth noting that there is currently no screening facility for HEV in any blood establishment.</p> Enayatullah Hashemi Akhlaaq Wazeer Noore Saba Zahida Qasim Aurooj Fatima Jahangir Anjum Usman Waheed Raja Tahir Mahmood Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 3 6 Judicious Transfusion of Fresh Frozen Plasma <p><strong>Background:</strong> Fresh Frozen plasma is prepared by centrifugation of whole blood or apheresis donation. It contains clotting factors, fibrinogen, anticoagulants, and certain electrolytes. FFPs are used worldwide for various indications, but their usage is mainly governed by clinical discernment rather than scientifically proven indications. Injudicious use of FFPs is not only a burden on resources but can also lead to adverse effects such as immune and non-immune mediated reactions, infections, and cardiopulmonary complications.</p> <p><strong>Objective:</strong> The objective of this study was to assess the judicious transfusion of fresh frozen plasma.</p> <p><strong>Methodology:</strong> After approval from the Aga Khan University Ethics Review Committee, medical record numbers for patients transfused with FFP's between the period October 2021 and December 2021 were extracted. Details including demography, reason for transfusion, admitting department and coagulation profile were acquired for every patient. Transfusion indications were decided as appropriate or inappropriate in view of British Committee for the Standards in Haematology (BCSH) guidelines. Data was then analyzed in SPSS v26.</p> <p><strong>Results:</strong> A total number of 310 patients were transfused FFPs between the study period, which accounted for a total of 1201 units. Age was distributed between 0 days to 81 years. Majority of the transfusions were performed by the department of Gastroenterology (14.2%), whereas adults and pediatric critical care contributed 11.6% and 8.1% respectively. Major diagnosis included sepsis (27.7%), malignancy (17.7%) and liver disease (17.4%). Most common indication revealed was deranged INR with active bleeding (28.4%). Out of the total transfusions, 68.4% of the transfusions were appropriate, leaving out 31.6% to be without a strong indication.</p> <p><strong>Conclusion:</strong> This analysis revealed a lack in judicious transfusion of fresh frozen plasma. These results can urge clinicians to make decision wisely for transfusion on the basis of research-based guidelines and evidence.</p> Harem Alam Natasha Ali Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 7 11 Effect of Platelet Functions Studies during 3 Days of Storage of Single Donor Platelets Obtained From Platelet-Apheresis <p><strong>Purpose: </strong>Platelet apheresis is a method to extract platelet from the whole blood in a donor. These helps in the blood coagulation but their storage condition could hinder their proper function after 72hour of storage.</p> <p><strong>Methodology: </strong></p> <p>This study was conducted at department of Hematology Children Hospital Lahore after obtaining informed consent from the donor by a trained staff member. Samples were chosen irrespective of gender and with the exact age of the cases. All the sample who have &lt;250,000/ µL platelet count were rejected. 92 sample were taken based on the WHO sample size estimated.</p> <p>Platelet concentration, aggregation was evaluated using different concentrations of inducing agonists: ADP (7.0 µg/mL). For each test, 400 µL of platelet rich plasma was used, each one in a different cuvette after waiting for spontaneous aggregation. The aggregation curve was observed after five minutes of stimulation by inducing agonists<sup>10</sup>. Chrono-log aggregometer was used for aggregation studies at 0day and 3<sup>rd</sup> day. SPSS was used for data analysis.</p> <p><strong>&nbsp;</strong></p> <p><strong>&nbsp;</strong></p> <p><strong>Result:</strong></p> <p>The average age of donors was 29.38(yr). More then half were female donor. Mean platelet count was 270300 µL. When aggregation of the platelet count was evaluated, it was found that 24(26.1%) have aggravated platelet at day 0. While on day 3, platelet aggregation was fond in 11(12%) of cases The percentage decrease in aggregation of platelet was 49(53.3%).</p> <p><strong>Conclusion:</strong></p> <p>There is reduction in platelet aggregation in the patients when they were evaluated at day0 and day 3.</p> Aiza Asghar Nida Ayub Ayesha Khanum Amna Shoukat Amaar Talib Ameena Nasir Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-13 2023-07-13 3 1 Diagnostic Accuracy of Haematology Analyzer Sysmex Xn-1000 Scattergrams in the Diagnosis of Malaria <p><strong><u>ABSTRACT</u></strong></p> <p><strong><em>Introduction: </em></strong>Malaria is a life threatening disease of tropical region caused by infection with plasmodium transmitted through the bite of an infected anopheles mosquito. Accurate malaria diagnosis is of principal importance and is critical in its treatment and management. At present, many alternative methods are in use for diagnosis such as rapid diagnostic tests rapid diagnostic tests&nbsp;and polymerase chain reaction. Microscopy is gold standard for malaria diagnosis by detecting plasmodium species.</p> <p><strong><em>Objective:</em></strong> To assess the diagnostic accuracy of automated haematology analyser Sysmex XN-1000 in the diagnosis of malaria taking microscopy as gold standard.</p> <p><strong><em>Materials and Methods</em></strong></p> <p><strong><em>Study design: </em></strong>Cross-sectional study<strong> &nbsp;</strong></p> <p><strong><em>Setting:</em></strong><strong>&nbsp; </strong>Haematology department Chughtai Institute of Pathology</p> <p><strong><em>Duration:</em></strong> Six months i.e. 26-4-2018 to 26-10-2018</p> <p><strong><em>Data collection procedure:</em></strong> Patients were segregated for being suspicion for positivity of malaria among the cases presenting with complaint of episodes of fever. Blood sample was evaluated for presence of any microbial activity. There were two tests, which were performed on the blood sample. First the auto analyser was applied. The other sample was run through microscopic method of analysis of plasmodium and under microscope. Results were recorded and entered for further analysis.</p> <p><strong><em>Results:</em></strong> Mean age of patients in this study was 41.21±13.77 years. Among patients 96(53.3%) were male and 84(46.7%) were females. Mean duration of symptoms was 4.01±1.99. Sensitivity and specificity of Sysmex Xn-1000 was 93.16% and 93.65% respectively and PPV and NPV was 96.46% and 88.06% respectively, overall diagnostic accuracy was 93.33%.</p> <p><strong><em>Conclusion: </em></strong>The diagnostic accuracy of automated haematology analyser sysmexXN-1000 for diagnosis of malaria was 93.33%. % so it may be concluded that the Sysmex XN-1000 analyser has an advantage in that it is able to detect unexpected malaria cases.</p> <p><strong><em>Key words: </em></strong>Diagnostic accuracy, automated haematology analyser, sysmexXN-1000, malaria, microscopy</p> Ghazala Qamar Ayisha Imran Imran Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 12 16 Comparison of Platelet Count by Automated and Manual Methods in Thrombocytopenia Patients <p><strong>Objective:</strong> This study aimed to investigate platelet count variations in adult thrombocytopenia patients by comparing automated platelet counts with manual counting methods.</p> <p><strong>Methodology:</strong> A comparative cross-sectional study was conducted at the Hematology department of King Edward Medical University, Lahore from January 2022 to June 2022, involving 60 patients with thrombocytopenia. Complete blood counts were performed using an Automated Hematology Analyzer, and peripheral smears were prepared and manually examined by two experts to verify platelet counts.</p> <p><strong>Results:</strong> The study included 31 females and 29 males, with a mean age of 43.7 years. The mean platelet count obtained from automated analyzers was 58 ± 28×10^9/L, while the manually verified platelet count on peripheral smears was 117 ± 13×10^9/L, with a significant p-value of &lt;0.001. Pseudo-thrombocytopenia was observed in 52% of patients, primarily due to platelet clumps (42%) and giant platelets (39%).</p> <p><strong>Conclusion:</strong> The study underscores the importance of manual verification of platelet counts in thrombocytopenic patients, as automated counts tended to underestimate platelet levels. Peripheral smears remain the gold standard for accurate platelet counting, helping prevent unnecessary investigations and ensuring appropriate patient care</p> <p><strong>Key words:</strong> Diagnostic accuracy, automated haematology analyser, sysmexXN-1000, malaria, microscopy.</p> Ayesha Tariq Arsala Rashid Hamda Khalid Sibgha Abdul Latif Sana Ilyas Muhammad Nouman Ria Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 17 20 Transfusion-Transmitted Hepatitis E: Implications for Blood Donation Screening <p>In places with inadequate sanitation, hepatitis E, which is caused by infection with the hepatitis E virus (HEV), is a frequent cause of acute hepatitis. HEV belongs to the Hepeviridae family, genus Orthohepevirus, and is an RNA virus. HEV is a new infectious threat to the safety of blood. Over the past 18 years, cases of HEV acquired by transfusions have been described. Concern over transfusion-transmitted HEV as a new global health issue is increasing. Immunocompromised people who get HEV may develop a persistent infection, increasing their risk of developing liver cirrhosis and even the inevitability of death. At even relatively low blood levels of the virus, HEV is contagious. The usefulness of HEV testing on all blood donors is still up for debate. Some countries have implemented universal screening of HEV&nbsp;after taking risk and resource availability into account. The key approach for prevention is the HEV NAT screening. Alternative approaches, such as NAT testing all or portion of blood donations individually or in a small pool, are being investigated. Future research is required to characterize the incidence of HEV&nbsp;transmission by transfusion as well as its clinical characteristics, prognosis, and consequences. This article reviews the available data on transfusion-transmitted HEV, summarizes the prevalence of HEV infections among blood donors, and discusses the significance of these findings for blood donor screening.</p> <p>&nbsp;</p> Akhlaaq Wazeer Usman Waheed Noore Saba Zahida Qasim Saeed Ahmed Raja Tahir Mahmood Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 26 33 Treatment Free Remission in Chronic Myeloid Leukemia- A Practical Option <p>Chronic myeloid leukemia (CML) is a type of blood malignancy. Unlike some other diseases and cancers, the treatment for CML typically spans many years. Patients achieving and sustaining a deep molecular response may be candidates for treatment stoppage. This concept also known as treatment free remission (TFR) is a new concept in CML management and of late has become a major goal. TFR has also taken the center stage owing to its implications in reducing health and financial burden being imposed by the disease. Numerous clinical trials have made an attempt to study as to which patients are the best candidates for treatment free remission and what percentage of such patients either stay in remission stage or face relapse. Findings of the trials suggest around 45-60% of patients being able to achieve TFR whereas the relapse mostly occurred in first six months of treatment stoppage. Nevertheless, almost all patients who were re-initiated on Tyrosine kinase inhibitors (TKIs) showed molecular response. Certain clinical and molecular biomarkers are thought to be linked to increase probability of TFR. In this review we attempt to discuss both clinical and molecular markers associated with TFR in CML.</p> Kaleem Ahmed Raheel Iftikhar Zafar Iqbal Muhammad Arshad Aleem Ahmed Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 34 42 A case of Renal Artery Thrombosis in Thalassemia Major Patient <p>Thalassemia, an inherited haemoglobin disorder, is the most common hemoglobinopathy in the world. Despite all the recent advances in the medical field to understand its pathophysiology, the management of thalassemia remains a complex process. Thalassemia patients suffer from many complications including the thromboembolic events. More commonly occurring thromboembolic complications include deep venous thrombosis (DVT), portal vein thrombosis, pulmonary embolism, and stroke. However, the renal artery thrombosis has never been reported. In this report we share a case of renal artery thrombosis in a thalassemia major patient.</p> <p>&nbsp;</p> <p>&nbsp;</p> Hamid Aizaz Chatha Mohammad Zadran Thiagarajan Jaiganesh Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 CHILDHOOD MYELODYSPLASTIC SYNDROME : A RARE ENTITY <p><strong>ABSTRACT :</strong></p> <p>Myelodysplastic syndrome(MDS) is a spectrum of bone marrow disorders resulting from clonal stem cell defect that manifest as cytopenias, ineffective hematopoiesis and dysplasia in all the three cell lines. Myelodysplastic syndrome is rare in children and accounts for less than 5% of the hematopoieitic malignancies below the age of 14 years. Refractory cytopenia of childhood (RCC) is the most common type of MDS that is seen in children. In this study, we report a case of &nbsp;14 years old boy who presented to us with suspicion of aplastic anemia and was diagnosed as refractory cytopenia of childhood, a type of myelodysplastic syndrome.</p> <p>Keywords: <em>Myelodysplastic syndrome, cytopenias, dysplasia, refractory cytopenia of childhood</em></p> Mavra Fatima Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 Successful outcome of pregnancy in Bernard -Soulier Syndrome with temporary endovascular balloon occlusion of uterine arteries: A Case Report <p>Bernard-Soulier Syndrome (BSS) is a rare congenital bleeding disorder, mainly inherited in an autosomal recessive pattern.The phenotype of BSS varies widely depending on particular mutation.Pregnancy in BSS is associated with a high risk of bleeding for mother and neonate, and there is no consensus about ideal management of these patients.</p> <p>AIM</p> <p>Use of perioperative endovascular balloon occlusion of internal iliac arteries (uterine artery), a minimally invasive technique that reduces blood loss and transfusion requirement in high bleedingrisk patients like BSS.</p> <p>Case Presentation</p> <p>It describes a successful pregnancy outcome with endovascular intervention in a woman with BSS who was closely monitored in pregnancy,peripartum, postpartum period,and had a planned birth.</p> <p>Conclusion</p> <p>&nbsp;Minimally invasive and effective endovascular prophylactic measures against bleeding reduces blood loss, transfusion requirement, platelet refractoriness and hospital stay during caesarean section in BSS</p> Maria Riaz Razia Bahadur Khero Muhammad yousaf Memon Muhammad Shahzad Sarwar Rozina Ghani Muhammad Farhan Muhammad Nadeem Parvez Ahmed Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1 A CHALLENGING CASE OF BETA-THALASSEMIA MAJOR WITH INCREASED RING SIDEROBLASTS IN BONE MARROW <p>Symptomatic Beta-thalassemia is one of the most common genetic disorders worldwide. Early clinical manifestations are variable. Routine hematologic analysis is usually sufficient to diagnose the disease, but sometimes diagnosis can be more difficult. We present the case of a 1-year-old male patient who underwent a bone marrow biopsy with clinical signs of inherited bone marrow/erythropoietic failure. Numerous ringed sideroblasts were found in iron-stained smears raising the suspicion of congenital sideroblastic anemia. The child was later genetically diagnosed as beta-thalassemia major.</p> Maha Tariq Sundas Ali Farah Hanif Rabiah Asghar Copyright (c) 2023 Journal of Haematology and Stem Cell Research 2023-07-12 2023-07-12 3 1