Journal of Haematology and Stem Cell Research

Hematopoietic stem cell transplantation in Pakistan: Current landscape and future prospects

Hamza Khan, Muhammad Younas, Ghulam Hassam ud Din Tarrar — Tue, 06 Aug 2024 00:00:00 +0000

(not applicable as the article type is "letter to the editor")

Frequency of Deletion 13q14.3 and Its Impact on Outcome in Patients of Chronic Lymphocytic Leukemia; A Single-Centered Institutional Study from Pakistan

Tue, 06 Aug 2024 00:00:00 +0000

Objectives: To evaluate the frequency and prognostic significance of this mutation in the Pakistani population.

Methodology: A retrospective study was conducted at Aga Khan University Hospital, Karachi, Pakistan, from January 2015 to December 2022. A total of 150 patients of all ages, diagnosed with chronic lymphocytic leukemia (CLL) according to the National Cancer Institute Working Guidelines for CLL (lymphocytosis >5 x 10?/L, CD19+, CD5+, CD23+, CD20 weakly positive, and expression of either kappa or lambda light chains), who received treatment or follow-up at Aga Khan University Hospital, were included in the study. Hematological parameters and FISH study data were obtained from the hospital’s electronic records. Progression-free survival (PFS) and overall survival (OS) were determined using Kaplan-Meier analysis, and the effects of mutations detected via FISH on prognosis and outcomes in CLL patients were evaluated.

Results: The most common mutations in the patient sample were deletion 13q14.3 (27%, n=28 [out of 150]), deletion 11q22 (13%, n=10), trisomy 12 (7%, n=6), and TP53 mutations (6%, n=4). In total, 47% (n=99) of patients had no detectable mutations on FISH. Patients with a deletion 13q14.3 mutation had a higher mean progression-free survival (128.9 months, 95% CI: 114.4–143.5) compared to the overall patient sample (68.1 months, 95% CI: 31.5–68.4), as well as a longer mean overall survival (127.0 months, 95% CI: 112–141) compared to the overall patient sample (67.0 months, 95% CI: 44.9–73.6).

Conclusion: Our study demonstrates that deletion 13q14.3 is the most common mutation in Pakistani CLL patients and is associated with a better prognosis in this population

Comparison of Automated Erythrocyte Sedimentation Rate with Gold Standard Westergren Method and its Correlation with Red Cell Parameters

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To compare the automated ESR with the gold standard Westergren method and investigate its correlation with red cell parameters.

Methodology; A total of 400 randomly collected blood samples (15-78 years of age) from Armed Forces Institute of Pathology (AFIP) Rawalpindi were assayed parallelly using Westergren method and Greiner-Bio-One (Vacuette?) SRS 20/II ESR analyzer from October 2022 to January 2023. Results of these assays were analyzed using the Kolmogorov-Smirnov test for normality and Spearman's correlation test for correlation.

Results: This study revealed a strong and significant correlation between the automated ESR analyzer and the classical gold standard Westergren method (r=0.907; p < 0.001). Additionally, the red blood cell parameters showed a positive and significant correlation with both automated and Westergren methods (p < 0.001).

Conclusion: With strong correlation between Greiner-Bio-One (Vacuette?) SRS 20/II ESR automated analyzer and the reference Westergren method, this study concludes that the automated technique is substantially associated with the Westergren method for estimating ESR, and red blood cell parameters have a positive and significant correlation with both methods.

Acute Myeloid Leukemia: Correlation of NPM1 Mutation with Morphology and Immunophenotypic Findings

Tue, 06 Aug 2024 00:00:00 +0000

BACKGROUND: NPM1 mutation is the most commonly mutated leukemic entity in acute myeloid leukemia (AML) and usually poses a favourable prognosis. It is important to find an association of this mutation with clinical presentation, different hematological parameters and immunohistochemistry to assist the pathologists with stratification of AML cases having different prognostic subgroups in low resource settings where molecular analysis is not easily available for NPM1 detection.

OBJECTIVE: to correlate NPM1 mutation in acute myeloid leukemia with specific morphology and immunophenotyping in Pakistani population.

Material and methods: Clinical history with peripheral blood and bone marrow aspirate samples were collected from 50 newly diagnosed cases of AML at Chughtai Institute of pathology from June 2022 to April 2024 after taking informed consent. NPM1 mutation was detected using Imegen-NPM1 kit via Reverse Transcriptase-Polymerase Chain Reaction (PCR).

Statistical Analysis: Normality of data was assessed using Kolmogrov Smimov test. Comparison was made using Chi-square test.

Results: NPM1 mutation was detected in 20% of the AML cases affecting mainly female population of age 40 years and above. Relatively high TLC count, M4 FAB type morphology and low CD34 expression (p<0.05) was observed in NPM1 mutated cases. 1 year remission was seen in 60% of the cases diagnosed with NPM1 suggesting a significant correlation between NPM1 mutation and remission (p<0.05).

Conclusion: NPM1 mutation is less common in our society and mainly correlates with female gender, old age, certain hematologic and immunhistochemical markers as well as with better overall remission.

Key Words: Acute myeloid leukemia (AML), Nucleophosmin1 (NPM1) gene, Hematologic and immunophenotypic parameters.

Epidemiological Profile of Patients with Hemophilia in Northern Pakistan: A Single-Center Study

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To study the epidemiological profile of patients with haemophilia in northern Pakistan.

Methodology: This research was cross-sectional and observational. Investigations were conducted on hemophiliac patients of various ages who had registered with the Hemophilia Patients Welfare Society in Rawalpindi. A thorough clinical and epidemiological history was gathered from the patient or the patient's accompanying parent or guardian using a pre-structured questionnaire. In accordance with test results and factor VIII and IX assay levels, patients were further divided into mild, moderate, and severe groups.

Results: One female patient of Hemophilia B is one of the important findings of this study. 0ut of total 837 patients investigated, 702 were of Hemophilia A and 135 were of Hemophilia B. 46.47% (389/837) belongs to the age group of >17 years. 476 (56.86%) were severe hemophiliacs, 269 (32.15%) were moderate and 92 (bn 10.99%) were mild hemophilia patients. Knee was the most involved joint and it was observed in 331 (39.57%) patients.

Conclusion: The most common type of hemophilia is the severe form of hemophilia a (classic hemophilia). This study also found a female hemophilia B case which is the novel identification of this study. It is concluded that there is a need to start a national level diagnosis, prevention and care program for hemophilia patients.

Frequency of Thiopurine Methyltransferase Gene Polymorphism in Acute Lymphoblastic Leukemia

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To determine the frequency of TPMT gene polymorphism in patients presenting with ALL at NIBD.

Methodology: A cross sectional study was done at the department of Hematology National Institute of Blood Diseases (NIBD) Karachi, from 15th March 2021 to 15th September 2021. A total of 60 patients of either sex presenting within duration of 2 months of diagnosis of ALL were included in the study. Blood samples of all the patients were drawn by senior laboratory technician and sent for Cytogenetic study as well as polymerase chain reaction to identify different alleles of thiopurine methyltransferase gene. The data was entered and analyzed using SPSS version 21. Frequencies and percentages were calculated for categorical variables such as gender, drug toxicity, and allelic variation (TPMT).

Results: Age range in this study was from 14 to 60 years with mean age of 25.733±14.62 years and mean duration of disease was 1.56±0.49 months. Male patients were 80% and females were 20% in this study. Variant TPMT alleles were observed in 10% patients.

Conclusion: The study revealed a 10% frequency of major polymorphisms in 6-MP metabolizing enzymes among Pakistani patients with ALL.

Mean Platelet Volume in Patients of Acute Coronary Syndrome

Tue, 06 Aug 2024 00:00:00 +0000

Objective: The purpose of our study was to assess the accuracy of elevated mean platelet volume (MPV) as an independent diagnostic marker for acute coronary syndrome (ACS).

Methodology: This descriptive cross-sectional study was conducted in the Department of Hematology in collaboration with the Department of Cardiology (CCU) at Fauji Foundation Hospital Rawalpindi, from December 2017 to June 2018. The Coronary Care Unit (CCU) records of 100 ACS patients admitted to FFH Rawalpindi were reviewed. All collected data were entered into SPSS version 17. Mean and standard deviation were calculated for quantitative variables, such as MPV and age. An independent sample t-test was used to compare effect modifiers like age and gender, with statistical significance set at p<0.05.

Results: A total of 100 patients were enrolled, with a mean age of 57.06±9.95 years for males and 56.0±9.48 years for females. There were 17 (17%) male and 83 (83%) female patients. The mean platelet volume was found to be higher among ACS patients (10.736±0.89 fl). The mean MPV in patients aged ?55 years was 10.63±0.97, while in patients aged >55 years it was 10.82±0.81. The mean MPV for male patients was 10.44±0.72, and for female patients, it was 10.80±0.91. No significant difference was observed in mean MPV between genders or age groups (p>0.05).

Conclusion: The study concluded that MPV significantly increases in patients with acute coronary syndrome and may serve as a potential diagnostic marker in such patients.

Frequency of Factor V Leiden in Patients Presenting With Venous Thromboembolism

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To determine the frequency of FVL mutation in patients of VTE presenting with or without predisposing environmental factors.

Methodology: It was a cross-sectional study conducted at Chughtai Institute of Pathology, from August 2022 to June 2023. The study included 80 adult patients aged 15 years and above and both genders, who were recently diagnosed with venous thromboembolism at any site in the body through radiological investigations (colored Doppler ultrasound) and put on anticoagulants. Data was collected from 80 diagnosed cases of VTE after informed consent over a period of 10 months. 5ml Blood sample in EDTA vial was analyzed for FVL using real time PCR after extracting DNA via kit method. The results were correlated with the clinical history and recurrence of the disease. Cases of VTE with or without any predisposing environmental factor and who were recently put on anticoagulant therapy were included in the study.

Results: FVL mutation was detected in 22.5% of the sample population affecting 18 individuals out of 80 patients with heterozygous mutation being more common. Recurrence was observed with FVL mutation suggesting significant association of recurrence with mutation (p value <0.05).

Conclusion: FVL mutation was found to be one of the common causes of VTE being identified in >20% of the population under study and was more likely associated with the recurrence of the disease in patients once their anticoagulants were put on hold.

Pre-analytical Factors Affecting Cytogenetic Cell Culture Yield in Haematological Malignancies

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To determine the association between different factors and the yield of cytogenetic culture in-terms of failure in samples drawn from patients suffering from haematological diseases.

Methodology: This descriptive cross-sectional study was conducted in Department of Haematology, Armed Forces Institute of Pathology, Rawalpindi from Dec 2021 to Sep 2022. Two hundred and twenty six peripheral blood and bone aspirate samples received for cytogenetic culture. Samples from patients aged between 5 and 70 years, of both genders, suffering from a primary haematological disease were included.

Results: The sample had a median age of 34.5 (7 - 64) years, of whom 123 (54.4%) were male. Samples with culture failure had significantly lower volumes of receipt with a mean value of 2.10 ± 0.98 mL versus 3.52 ± 0.99 mL in cultures that were successful, (p<0.001). Samples that were partially clotted also had a higher incidence of cell culture failure, (p<0.001), with only 8.8% of samples showing clotting in those that were successful versus 26.9% that had cell culture failure. Additionally, cell culture had a higher chance of yielding a successful result if it was received for processing within twenty-four hours of withdrawal, with the success rate decreasing with the passage of time especially past the seventy-two-hour window, (p<0.001).

Conclusion: Delayed sample dispatch, clotted samples and inadequate volume are important factors associated with the failure of cell culture for cytogenetics

Clinical Audit of Bone Marrow Examination - Suggesting the Impact of Procedural Checklist to Improve its Quality

Omer Javed, Bushra Kaleem, Esha Farooq, Fatima Meraj, Hamza Khan — Tue, 06 Aug 2024 00:00:00 +0000

Objective: The purpose of bone marrow biopsy includes diagnosis, staging, prognosis, and treatment response for various hematological and non-hematological disorders. The present study aimed to observe the frequent indications for the performance of bone marrow biopsy and the frequencies of various disorders diagnosed using the procedure.

Methodology: This descriptive retrospective audit was conducted in the Hematology section at Indus Hospital and Health Network and included bone marrow biopsies performed from January 2021 to October 2022 with patients >16 years and belonging to either gender. Bone marrow trephine biopsy was performed using Jamshidi needle from posterior superior iliac spine while aspiration was performed from same site using a spinal needle.

Results: A total of 111 bone marrow biopsy procedures were performed. The procedure was performed with ease in 68.4% of the cases by competent residents. In 27.9% cases procedure was performed with difficulty due to patient related causes which included immobility, displaying non-cooperation or irritability, or difficulty in the localization of the bone. Maximum diagnosis ( 63.1%) was made when the core depth was >1.0 cm. Hematological diseases were the most common diagnosis (69.4%); of which the majority were malignant (70.1%)in almost half of the cases. Concordance was present between the suspected diagnosis and the conclusive diagnosis made in the bone marrow biopsy report. Bone marrow was found to be normal in 13.5% (n=15) patients. Chronic myeloid leukemia (n=17; 22.1%) and aplastic anemia (n=9; 11.7%) were the most frequent malignant and benign disorders respectively. The overall diagnostic yield for the present study was 82.9% while based on indication, visceromegaly produced a yield of 87.1%.

Conclusion: Splenomegaly was observed to be the most common indication for procedure referrals in our center. Core size of >1.0 cm was found to have the highest diagnostic utility. Chronic Myeloid Leukemia and aplastic anemia were the most frequent hematological diagnoses in the present study

Immature Platelet Fraction: A Novel Hematological Parameter for Predicting Prognosis in Dengue Patients

Tue, 06 Aug 2024 00:00:00 +0000

Objective: Therefore, the aim of our study is to evaluate IPF as an early recovery indicator of platelet count in dengue patients with thrombocytopenia.

Methodology: This study was conducted at Chughtai Institute of Pathology, Lahore from October 2022 till December 2023. A total of 199 patients, with NS1 antigen were included in the study. Immature platelet fraction (IPF) and total platelet count were measured by Sysmex XN 1000 on day 1 and day 5 of the infection. IPF and its co relation with platelet count on day 1 and day 5 of infection was analyzed. Data was analyzed by using SPSS version 23.00. Paired sample t-Test was used to assess correlation between the two main variables.

Results: Out of 199 patients, 104 (52.3%) were males and 95 (47.7%) were females. Mean age of the patients was 39 years. There was a strong correlation between IPF values and the recovery of total platelets. IPF values tended to be higher at low values of platelet counts (p<0.001) at Day 1 whereas IPF decreased on Day 5 with an increase in platelet count showing statistically significant correlation

Conclusion: IPF is a good prognostic marker for recovery of platelets in DENV infection. It is a reliable and cost-effective tool that can help clinicians avoid unnecessary platelet transfusion.

Red Blood Cell Indices and Morphology in the 3rd Trimester of Pregnant Women Attending Isra University Hospital

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To calculate RBC indices in third trimester of pregnancy among anemic and non-anemic women and to observe RBC morphology on peripheral blood smear.

Methodology: A cross-sectional comparative analysis was done at department of Obstetrics and Gynecology, Isra University Hospital Hyderabad, Sindh, from March 2019 to August 2019. All the gravid women aged 25 to 50 years old with 3rd trimester of gestation (more than 28 weeks of gestation) were included. Approximately 5 mL of blood was drawn into EDTA tubes. RBC indices, including hemoglobin concentration (Hb), hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), and mean corpuscular hemoglobin concentration (MCHC), were measured using an automated hematology analyzer. Peripheral blood smears were prepared from the collected blood samples and stained. The stained smears were examined under a light microscope by a qualified hematologist and the morphology of RBCs were assessed. Analysis of data was done using SPSS version 26.0.

Results: Study compared the red blood cell indices and morphology in the 3rd trimester of pregnant anemic and non-anemic women within a sample of 100 participants. Group A (non-anemic) had significantly higher mean Hb levels (11.6 ± 2.1 g/dL) compared to Group B (anemic) (7.7 ± 1.8 g/dL), with a p-value of 0.001. Mean hematocrit was significantly higher in Group A (36.8 ± 9.1%) than in Group B (30.3 ± 9.6%), and the mean RBC count (millions/µL) was also higher in Group A (3.49 ± 0.04) compared to Group B (2.82 ± 0.47), both with a p-value of 0.001. Further analysis showed that Group A had higher mean values for MCV (83.11 ± 11.26 fL vs. 64.56 ± 7.55 fL), mean MCH (28.28 ± 4.54 pg vs. 22.73 ± 4.28 pg), and mean MCHC (30.57 ± 4.33% vs. 23.32 ± 4.53%) compared to Group B, (p-0.0001) for all comparisons. Conversely, mean RDW was higher in Group B (15.13 ± 2.9%) than in Group A (11.20 ± 2.5%), (p-0.001).

Conclusion: The study revealed a high prevalence of anemia (65%) in the third trimester of pregnancy, characterized by significant decreases in RBC indices such as MCV) and MCH. Additionally, elevated RDW levels, even with normal MCV, is an indicator of iron deficiency anemia, especially during pregnancy

The Effects of Iron Therapy on HbA2 Levels in Patients with Iron Deficiency Anaemia; A Study in Khyber Pakhtunkhwa

Khush Bakht, Yasar Mehmood Yousafzai, Raza Muhammad Khan — Tue, 06 Aug 2024 00:00:00 +0000

Objectives: This study aimed to determine the effect of iron treatment in a post-therapy group of patients with Iron Deficiency Anemia (IDA) in the population of Khyber Pakhtunkhwa (KPK).

Methodology: This quasi-interventional study was conducted from January 2021 to January 2022 at Hayatabad Medical Complex (HMC) and the Institute of Pathology and Diagnostic Medicine, Hematology Laboratory. The participants were aged 8–15 years. To assess the response to iron therapy in both pre- and post-therapy iron-deficient groups, an Electronic Particle Counter (EPC) was used to measure Mean Cell Volume (MCV), and a chemiluminescence immunoassay was used to measure serum ferritin levels in biofluids. SPSS version 19.0 was used for data analysis. A paired T-test was performed for the mean/SD of all hematological parameters recorded before and after iron treatment. Pearson correlation was used to determine the strength of the association between pre- and post-therapy hematological parameters. The level of significance was set at P<0.05.

Results: The mean age of the participants was 9.5±3.5 years, with a male-to-female ratio of 2:1.5. A positive correlation was found between pre- and post-therapy hemoglobin levels (Hb1 and Hb2; r = 0.851) and between pre- and post-therapy MCV values (r = 0.806). The mean MCV before treatment was 55.30±7.42 fL, which increased significantly to 75.82±4.66 fL by day 180 (P<0.001). Similarly, the mean hemoglobin level, which was initially 9.22±1.17 g/dL, increased to 11.22±1.21 g/dL by day 180 (6 months) with a high level of significance (P<0.001). The greatest difference between pre- and post-treatment was observed in ferritin levels (19.66±6.79 ng/mL vs. 62.48±4.82 ng/mL, P<0.001). There was a statistically significant increase in hemoglobin levels (P<0.001), MCV (P<0.001), serum ferritin (P<0.001), and hemoglobin A2 levels.

Conclusion: The study demonstrated statistically significant improvements in hematological parameters following iron therapy, both orally and intravenously, validating the effectiveness of iron treatment in IDA patients.

Determination of Mean HbA2 Levels by High-Performance Liquid Chromatography in Individuals with Known Beta Thalassemia Trait

Tue, 06 Aug 2024 00:00:00 +0000

Background: Pakistan bears the largest burden of thalassemia affecting 5-7% of its population. Patients with aberrations in the beta globin chain are beta thalassemics which are further classified as trait, intermedia and major. HPLC is considered as a technique of choice for screening thalassemias due to its reliability, speed and sensitivity.

Objective: The present study was conducted to determine mean HbA2 levels of known ? thalassaemia trait individuals on high performance liquid chromatography.

Methodology: A descriptive cross-sectional study was conducted from November 2018 to April 2019. Thirty patients who were diagnosed as ? thalassaemia trait on cellulose acetate hemoglobin electrophoresis were included in the study. The blood samples were run-on High-Performance Liquid Chromatography (HPLC) and HbA2 levels were recorded on the proforma. Mean and standard deviation were calculated for HbA2, MCV and MCH. Frequency and percentage were calculated for gender. Effect modifiers like age and gender were controlled by stratification and post stratification independent sample t test was applied.

Results: Mean HbA2 levels of ?thalassaemia trait individuals on HPLC was 5.63%. There was no effect of age (p=0.07) and gender (p=0.14) on mean HbA2 levels. Mean Hb was 9.6g/dl. Mean corpuscular volume (MCV) and Mean corpuscular haemoglobin (MCH) were reduced with mean value of 56.7 fl and 18.5 pg respectively.

Conclusion: HPLC is a fast, precise, and reliable method for the early detection and management of hemoglobinopathies and their variants especially beta thalasemia trait. Raising awareness about thalassemia and its relatively simple prevention is crucial for the success of a thalassemia control program.

Donor Variables as Major Determinants of Platelet Yield in Plateletpheresis

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To identify the major donor variables that can affect the platelet yield so that improvement in donor selection criteria can be suggested.

Methodology: This is a cross-sectional study conducted at the Department of Hematology, Chughtai Institute of Pathology May 2022 to May 2023. A total of 116 male donors were recruited in the study. The procedure of plateletpheresis was performed on Cobe spectra, Fresenius Kabi, Trima Accel or Spectra Optica. Platelet yield was calculated and data was analyzed using SPSS 23.00. Relationship between continuous donor variables and platelet yield was studied by using Pearson coefficient. For categorical donor variables one way ANOVA test was used. A p-value < 0.05 was considered significant.

Results: No significant correlation was seen between platelet yield and donor´s age, weight, hemoglobin levels, blood group and Rh factor. However, a significant correlation was observed between total pre-donation platelet count of the donor and platelet yield.

Conclusion: While factors like age, weight, and blood group showed no significant correlation, pre-procedure platelet count strongly influenced platelet yield. Donors with higher platelet counts yielded more platelets, emphasizing the importance of donor selection based on platelet count.

Blood Donor Deferral Patterns and Frequency at a Tertiary Care Hospital in Lahore

Tue, 06 Aug 2024 00:00:00 +0000

Objectives: To determine the frequency and the common causes of donor deferral in blood bank of Shalamar hospital Lahore.

Methodology: Retrospective study was conducted at blood bank of Shalamar Hospital Lahore from Jan 2020 to December 2021. The data of blood donors who were registered in the blood bank during the mentioned period were reviewed. As per mandatory WHO guidelines eligible donors were screened for Hemoglobin and Transfusion Transmitted Infection (HBsAg, HCV antibody, HIV 1/2 antibody, Syphilis and Malaria). The screening data of donor was analyzed in order to determine the frequency and most common causes of deferral using SPSS version 19.

Results: Among 6616 blood donors who registered in blood bank during mentioned period, donor deferral rate was 4.2%. The mean age of deferred individuals was 31.39 ±8.0 years. The most frequent cause of temporary deferral was Low Hb (32.8 %) followed by Syphilis detected in 20.6% of donors .Hepatitis C Virus infection ( 25.5%) was leading cause of deferral among permanently deferred donors followed by Hepatitis B virus (18.8%) and HIV (2.3%). No positive case of malaria was detected in this study.

Conclusion: Deferral for blood donations accounts for 4.1%. Low Hb and HCV infection were the two most common causes of deferral observed in present study. The substantial rate of deferral due to anemia and HCV infection poses a significant public health concern, necessitating regulatory bodies to implement measures at national level aimed at enhancing community health and minimizing infection rates by public awareness and strict regulatory policy.

Frequency of A1 and A2 Subtype of Blood Group A and AB

Tue, 06 Aug 2024 00:00:00 +0000

Objective: To assess the frequency of A1 and A2 subtype of blood group A and AB in patients presenting in a tertiary care hospital

Methodology: This Cross sectional study was conducted at Department of hematology, Lahore General Hospital, Lahore for 6 months i.e. 3-3-2022 to 3-9-2022. Total 235 candidates fulfilling selection criteria were selected. Using a 5cc disposable syringe and aseptic procedures, blood samples were taken and then preserved in sterile EDTA vials. The blood groups A and AB were verified using the standard ABO blood group procedure. To further differentiate between A1 and A2, samples were analysed using anti-A1 lectin. A1 was taken into consideration if agglutination with anti-A lectin occurred. The sample was classified as an A2 subgroup if the agglutination was 4+ with anti-A antisera but negative with anti-A1 lectin.

Results: The mean age of the patients was 18.31±6.54 years. There were 153(65.1%) males and 82(34.9%) females in this study. There were 207(88.1%) patients and 28(11.9%) donors in this study. There were 180(76.6%) patients and donors with blood group A and 55(23.4%) with blood group AB. Out of total 235 subjects of blood group A and AB, 184(78.3%) participants present with subtype A1 and 51(21.7%) with subtype A2. A1 is the main subgroup of the blood groups A and AB. Subgroup A2 constitute a significiant percentage of blood group A and AB.

Conclusion: It is recommended that in all blood banks anti sera A1 and A2 be used for subgrouping blood group A.

Physiological Implications of Neutrophil-to-Lymphocyte Ratio in Chronic Obstructive Pulmonary Disease

Tue, 06 Aug 2024 00:00:00 +0000

Objectives: To determine the physiological significance of Neutrophil-to-Lymphocyte Ratio in inflammatory responses and clinical outcomes of COPD.

Methodology: A case control study that was conducted at Medicine Department of Muhammad Medical College Mirpurkhas, Pakistan, from April 2022 to June 2022. All the patients aged 40 years and above, both gender, clinically diagnosed as patients of COPD and healthy individuals without pulmonary or systemic inflammatory conditions (controls) were included. A 5ml blood sample was obtained from each case and immediately was send to diagnostic laboratory for complete blood count test to assess the neutrophils and lymphocytes. Data was analyzed using GraphPad Prism 9 software, with statistical significance determined by Fischer’s exact test (p ? 0.05).

Results: The average age of the case group (n = 150) was 59 years, while the control group was around 53 years old. 83.33% of the COPD participants had an NLR greater than 3, and 16.67% had an NLR less than 3. The p-value of 0.0001 indicates a statistically significant difference between the groups. The odds ratio was 0.07027.

Conclusion: A significant relationship was observed between an elevated Neutrophil-to-Lymphocyte Ratio (NLR) and COPD, with COPD patients showing higher neutrophil levels compared to the general population. Easily evaluated and with strong association with disease progression, NLR can serve as an effective marker for routine clinical assessments in COPD patients.

Successful Pregnancies in a Case of Severe (Type 3) von Willebrand Disease

Tahira Zafar, Nadeem Ikram, Lubna Zafar, Raana Zeeshan, Asma Naseer — Thu, 08 Aug 2024 00:00:00 +0000

Given the wide heterogeneity of phenotypes and the underlying pathophysiological mechanisms associated with the disorder , pregnancy and delivery in VWD represents a significant clinical challenge . The variable pattern of changes observed during pregnancy in von willebrand factor (VWF) and factor VIII (F-VIII), the protein carried by VWF , prompts a careful evaluation of pregnant women with VWD to plan the most appropriate treatment at the time of parturition. ¹Women with von Willebrand Disease (VWD) are at an increased risk of bleeding and other complications during pregnancy and childbirth.^2,3Women with inherited bleeding disorders, including VWD, are concerned about their bleeding and possible bleeding complications. All these leads to upfront efforts to give this cohort a safe reproductive fruition .Last 20 years have witnessed progression in understanding diagnosis, and treating complications in women with VWD .^1-6

Pegylated L-Asparaginase Induced Cholestatic Jaundice and Treated With Oral L-Carnitine: A Case Report

Asma Nasir, Ayisha Imran, Tasneem Farzana — Tue, 06 Aug 2024 00:00:00 +0000

Various chemotherapeutic regimens are available for the treatment of acute lymphoblastic leukemia (ALL). While providing improved cure rates these regimens are also associated with various systemic toxicities including hepatotoxicity. L-Asparaginase is a commonly used drug in various combination regimens for the treatment of both adult and childhood ALL. Most commonly observed side effect is hypersensitivity followed by liver injury. 85% of all the patients treated with L-Asparaginase develop hepatic steatosis whereas Cholestatic jaundice is infrequently seen. Timely identification and management of this entity can prevent progression to fatal liver failure.We, here, describe a patient of acute lymphoblastic leukemia diagnosed on bone marrow biopsy and immunophenotyping who developed Cholestatic jaundice after induction chemotherapy using L-Asparaginase, Vincristine, Daunorubicin and Dexamethasone. He was identified earlier and was given a good supportive management along with holding chemotherapy. He recovered within a span of two weeks and is on maintenance chemotherapy doing well on last follow up.

Reproductive Life of Women With Inherited Bleeding Disorders

Nadeem Ikram, Tahira Zafar — Tue, 06 Aug 2024 00:00:00 +0000

From Inflammatory Cascades to Hematopoietic Consequences; A Review of Bone Marrow Failure Mechanisms

Shahzad Ali Jiskani — Tue, 06 Aug 2024 00:00:00 +0000

Hematopoiesis plays an essential role in supporting immune cell function and different physiological processes, such as nutrient transport, hemostasis, and would healing. In inflammatory scenarios, the typically stable state of hematopoiesis shifts to emergency myelopoiesis, generating necessary effector types of cell to address acute insults. Prolonged or unusual exposure of inflammatory signals adversely affects the hematopoiesis, causing enhanced proliferation, damage to DNA, and cell death such as necrosis, apoptosis, and pyroptosis. Additionally, the microenvironment of bone marrow undergoes alterations. Collectively, such variations can results in the early impairment of hematopoiesis. Particularly in patients having immune-mediated aplastic anemia or inherited bone marrow failure syndromes (BMFS), continuous exposure to inflammatory signals can worsen cytopenias and expedite the progression of disease. Nonetheless, the comprehension of specific characteristics of inflammation in bone marrow failure is understood poorly. This review synthesizes findings from diverse mouse models exploring inflammatory mechanisms in bone marrow failure and delves into their implications for prospective research and clinical applications.