Indian Journal of Hematology and Blood Transfusion最新文献

{"title":"Clinical Relevance of Interferon Regulatory Family-4 (IRF4) Expression in Newly Diagnosed Patients with Multiple Myeloma.","authors":"May E Abdelmonem,&nbsp;Hend A Nooh,&nbsp;Mona S El Ashry","doi":"10.1007/s12288-023-01628-3","DOIUrl":"https://doi.org/10.1007/s12288-023-01628-3","url":null,"abstract":"<p><p>Multiple myeloma (MM) is a malignant plasma cell neoplasm with complex biology and heterogenous course. Interferon regulatory factor 4 (IRF4) transcription factor, important key developmental stages of hematopoiesis, represents an excellent potential therapeutic target. The present work aimed to investigate the expression status of IRF4 in the diagnostic bone marrow biopsy (BMB) cores of MM patients. This prospective study included 62 newly diagnosed MM patients. The expression of IRF4 was assessed in the BMB by immunohistochemistry (IHC). The data were correlated to the patients' clinico-pathological features, response to treatment and survival rates. IRF4 expression was observed in 50% of MM patients (31/62). IRF-4 positive patients were more frequently male patients (<i>P</i> = 0.018), have immunoglobulin heavy chain (IgH) translocations (<i>P</i> = 0.05) and tended to present with a higher platelets count (<i>P</i> = 0.07). Multiple myeloma patients presenting with urine M-protein had worse overall survival (OS) than negative cases (<i>P</i> = 0.012). Normocellular BM aspirate (BMA) was associated with better OS than hypercellular and hypocellular BMA (<i>P</i> = 0.006). Patchy distribution of plasma cells in BMB was associated with better disease-free survival (DFS) while diffuse infiltration had the worst (<i>P</i> = 0.019). Of note, after treatment, MM patients had significantly lower percentage of BMA plasma cells, platelet count, <i>β2</i> microglobulin and creatinine levels (<i>P</i> = 0.037, < 0.001, 0.022 and 0.026, respectively). Had higher albumin level (<i>P</i> = 0.007), compared to initial investigations. No significant association was found between IRF4 expression and the patients'clinical outcomes. Patterns of plasma cells distribution in BMB, BMA cellularity and urine M-protein are prognostically relevant in MM.</p><p><strong>Supplementary information: </strong>The online version contains supplementary material available at 10.1007/s12288-023-01628-3.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41170468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Castleman Disease Variant of Poems Syndrome Complicated with Multiple Osteosclerotic Lesions.","authors":"Manaswinee Mallik,&nbsp;Lakshmon Kalikkaleth,&nbsp;Naresh Kumar Chirumamilla,&nbsp;Arihant Jain,&nbsp;Charanpreet Singh,&nbsp;Aditya Jandial,&nbsp;Amanjit Bal,&nbsp;Deepesh Lad,&nbsp;Alka Khadwal,&nbsp;Pankaj Malhotra,&nbsp;Gaurav Prakash","doi":"10.1007/s12288-023-01637-2","DOIUrl":"https://doi.org/10.1007/s12288-023-01637-2","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542060/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41180380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Study of the Frequency and Specificity of Red Cell Antibodies in Patients with Hemoglobinopathies.","authors":"Manal M Wilson,&nbsp;Manal M W El Masry,&nbsp;Mona Kamal El-Ghamrawy,&nbsp;Nessma Abd El-Hadi,&nbsp;Amany A Abou-Elalla","doi":"10.1007/s12288-023-01651-4","DOIUrl":"10.1007/s12288-023-01651-4","url":null,"abstract":"<p><p>Patients with thalassemia and sickle cell disease (SCD) require blood transfusions as part of their supportive care. However, one of the most serious side effects of this treatment is the risk of red cell alloimmunization. The goal of this study was to assess the prevalence and Specificity of red cell alloimmunization in Egyptian thalassemia and sickle cell anaemia patients. This study included 200 multi transfused Egyptian patients, one hundred and forty patients with transfusion dependent thalassaemia and sixty patients with sickle cell anaemia, who were attending the Paediatric Children Hospital-Cairo University at the period from March 2019 to October 2019. Alloantibody identification was made by Diamed- ID microtyping system. In the studied groups both thalassemia and sickle patients, the prevalence of alloimmunization was 22/200 (11%) patients. The two most often alloantibodies were, antibodies against Kell antigen (37%) and against E antigen (30%). The prevalence of alloimmunization was more in females in comparison to males, but it did not reach statistical significance and patients with thalassemia major had higher alloimmunization rates than other studied groups but was not statistically significant. In the D negative patients in the research group, alloimmunization demonstrated a statistically significant difference (<i>p</i> = 0.01). Age, gender, age of transfusion onset and splenectomy were not contributing factors to the antibody presence in the group of patients being investigated. Before receiving blood transfusions, extended red blood cell phenotyping should be thought of as a crucial procedure for hemoglobinopathies patients who would likely have several transfusions. It is advised that haemoglobinopathies patients in Egypt be checked through phenotyping of RBC units for Kell and all Rh antigens to be phenotyped before starting transfusion in these patients which is also standard of care for these patients presently.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542054/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41174343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparison of HbA2 Using High Performance Liquid Chromatography Versus Haemoglobin Capillary Zone Electrophoresis.","authors":"Gurpreet Kaur,&nbsp;Seema Tyagi,&nbsp;Tulika Seth,&nbsp;Manoranjan Mahapatra,&nbsp;Ganesh Kumar Viswananthan,&nbsp;Jasmita Dass,&nbsp;Rama Hariharan,&nbsp;Arijit Sen","doi":"10.1007/s12288-023-01648-z","DOIUrl":"https://doi.org/10.1007/s12288-023-01648-z","url":null,"abstract":"<p><p>Thalassemia is among the most common hereditary disorders in the world. Approximately 5% of the world's population are carriers of hemoglobinopathies, and 2.9% are carriers of beta thalassemia. Haemoglobin A2 (HbA2) constitutes less than 3% of the total hemoglobin (Hb) in adults, and the determination of Hb A2 levels is important to diagnose the beta thalassemia trait (BTT). In some cases, the level of HbA2 is not typically elevated, and some difficulties may arise in making the diagnosis. Cation exchange high-performance liquid chromatography (HPLC) and HbCZE (haemoglobin capillary zone electrophoresis) are considered acceptable methods to diagnose BTT, but these vary in their accuracy and cut-offs. In this study, we attempted to compare HbA2 values using two methods, HPLC and HbCZE, in 536 whole blood samples sent by physician-ordered hemoglobinopathy screening over two years. This included antenatal women, patients with anemia not responding to iron, and cases of familial screening where either a child or a sibling had been diagnosed with hemoglobinopathy or thalassemia. The performance characteristics of both machines were compared for the detection of the 5 most common hemoglobin variants: Hb A, HbF, HbS, Hb C, and HbE. On comparing the HbA2 values, the HPLC showed higher values for HbA2 as compared to HbCZE, while the HbF and HbS measurement agreement was good between both methods. Normal ranges and mean normal values of HbA2 differ between different methods and different manufacturers; hence, each institute using these machines should validate its cutoffs.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542033/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41140423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Comparative Analysis of Early T-Cell Precursor Lymphoblastic Leukemia/Lymphoma (ETP-ALL/LBL) and Non-ETP-ALL/LBL in a Tertiary Cancer Care Center Based in Western India.","authors":"Anurag Saha,&nbsp;Beena Brahmbhatt,&nbsp;Varnika Rai,&nbsp;Sneha Kakoty,&nbsp;Jyoti Sawhney","doi":"10.1007/s12288-023-01627-4","DOIUrl":"https://doi.org/10.1007/s12288-023-01627-4","url":null,"abstract":"<p><p>Early T-cell precursor lymphoblastic leukemia (ETP-ALL) has a unique immunophenotype with very early T-cell differentiation. The current study summarises the distinct clinicopathological aspects of ETP-ALL and compares them with non-ETP-ALL. Twenty-nine ETP-ALL and 191 non-ETP-ALL cases were retrieved between 2018 and 2021. A <i>P</i> value was determined for each of the patient charaterisics (Table 1) to see for any significant relationship (<i>P</i> < 0.05) with ETP-ALL versus non-ETP-ALL. Kaplan-Meier log rank test was applied to look for any significant differences in OS for both the ALLs. ETP-ALL had an incidence of 12.6% out of total T-Acute lymphoblastic leukemia (T-ALL/LBL) in the past 3-years. Compared to non-ETP-ALL, ETP-ALL cases were associated with lower median age and male-to-female ratio. There was no statistically significant difference in the complete remission rate between both the subtypes. ETP-ALL was seen to be associated with high induction failure and relapse rate compared to non-ETP-ALL. To summarise, since the 2-year OS was poor compared to western research (for both ALLs), an intensive chemo-regimen should be implemented in the current situation. Some unusual markers were observed on flow-cytometry (ETP-ALL), which can be useful for MRD quantification, prognosis, and further trials for newer targeted therapies.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542041/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41154222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Co-inherited α-Globin Gene Cluster Duplication Compromises RBC Indices-Based Thalassemia Screening.","authors":"Huan-Qing Chen,&nbsp;Li-Sha Wu,&nbsp;Fan Jiang,&nbsp;Dong-Zhi Li","doi":"10.1007/s12288-022-01601-6","DOIUrl":"https://doi.org/10.1007/s12288-022-01601-6","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41155076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Conventional Gold Standard Techniques: Indeed a Saviour in the Era of Automation!!","authors":"Yashaswi Dhiman,&nbsp;Manish Raturi,&nbsp;Bhawana Adhikari,&nbsp;Himanshu Rawat,&nbsp;Dushyant Singh Gaur","doi":"10.1007/s12288-023-01644-3","DOIUrl":"https://doi.org/10.1007/s12288-023-01644-3","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41148705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thyroid Dysfunction in Patients with and Without Venous Thromboembolism: a Case Control Study.","authors":"Rizwana Naushad,&nbsp;Jayachandran Selvaraj,&nbsp;Jayaprakash Sahoo,&nbsp;Stalin Viswanathan,&nbsp;Rajeswari Murugesan","doi":"10.1007/s12288-023-01643-4","DOIUrl":"https://doi.org/10.1007/s12288-023-01643-4","url":null,"abstract":"<p><p>Recent studies report an association between thyroid dysfunction and venous thromboembolism (VTE). Considering the high prevalence of thyroid diseases in India, identification of thyroid dysfunction as a risk factor for VTE will have implications in management. The aim of study was to determine if thyroid dysfunction could be considered as risk factor for unprovoked VTE. The study was conducted on 102 patients with unprovoked VTE and 102 age and gender matched controls in a tertiary care centre. Clinical profile and thyroid function tests (Free T3, Free T4, TSH) including antibody profile (Anti TPO and Anti TG) were compared between two groups. Thyroid disease was identified in 34 cases and 14 controls (33.1% vs. 13.7%, <i>P</i> = 0.001) Out of 34 cases with thyroid dysfunction, 17 had subclinical hypothyroidism (SCH) while 6 out of 14 controls had SCH. Both thyroid dysfunction and SCH were found to be associated with unprovoked VTE, as compared with controls; [Odds ratio (OR) = 3.14, 95% CI 1.56-6.33, <i>P</i> = 0.001] and (OR = 3.71; 95% CI 1.4-9.9; <i>P</i> = 0.01) respectively. Thyroid dysfunction was significantly higher among patients with unprovoked VTE. Thyroid dysfunction, SCH were associated with unprovoked VTE.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542045/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41155077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Preconditioning Modified-Easix as a Predictor of Prognosis in Allogeneic Hematopoietic Stem Cell Transplant Recipients.","authors":"Zeynep Arzu Yegin,&nbsp;Emine Merve Savaş,&nbsp;Şeyma Yıldız,&nbsp;Münevver İrem Kök,&nbsp;Meltem Büşra Erdemir,&nbsp;Başak Bostankolu Değirmenci,&nbsp;Zübeyde Nur Özkurt,&nbsp;Münci Yağcı","doi":"10.1007/s12288-022-01623-0","DOIUrl":"https://doi.org/10.1007/s12288-022-01623-0","url":null,"abstract":"<p><p>Allogeneic hematopoietic stem cell transplantation (alloHCT) is associated with severe complications, most of which share a common physiopathological background characterized by endothelial dysfunction. A novel risk assessment model, endothelial activation and stress index (EASIX), has been introduced as a predictor of endothelial activation. This retrospective study was performed to evaluate the predictive impact of EASIX/modified-EASIX (mEASIX) on transplant outcome. Medical records of 398 alloHCT recipients [median age: 43(17-71) years; M/F: 243/155] were examined. EASIX/mEASIX were calculated at specific time points before and after transplantation. EASIX/mEASIX were significantly associated with transplant complications including engraftment syndrome, sinusoidal obstruction syndrome, febrile neutropenia and transplant associated thrombotic microangiopathy. The probability of overall survival was significantly higher in low-preconditioning mEASIX (day -7) group (37% vs 25.2%; <i>p</i> = 0.008; HR: 2.057; 95% CI: 1.208-3.504). The probabilities of day30 mortality (2.9% vs 19.4%; <i>p</i> = 0.017; HR: 7.028; 95% CI: 1.418-34.836), day100 mortality (9% vs 33%; <i>p</i> = 0.004; HR: 4.469; 95% CI: 1.619-12.336) and non relapse mortality (44.8% vs 61.4%; <i>p</i> = 0.005; HR: 2.551; 95% CI: 1.318-4.941) were lower in low-preconditioning mEASIX (day -7) group. This retrospective cohort analysis demonstrates the significant impact of EASIX/mEASIX on transplant complications and survival. Prospective analyses are mandatory to assess the predictive role of EASIX/mEASIX in clinical practice.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542067/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41160485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nonacog Alfa for Prophylaxis and Treatment of Bleeding Episodes in Previously Treated Patients with Moderately Severe or Severe Hemophilia B in India.","authors":"Nirmalkumar Choraria,&nbsp;Savita Rangarajan,&nbsp;M Joseph John,&nbsp;Shashikant Apte,&nbsp;Pritam Gupta,&nbsp;Seema Pai,&nbsp;Rohit Chand,&nbsp;Shyam Parvatini,&nbsp;G S H Ramakanth,&nbsp;Jeremy Rupon,&nbsp;Amit Chhabra,&nbsp;Hitesh Bhaskarrao Muley,&nbsp;Damien Simoneau","doi":"10.1007/s12288-022-01588-0","DOIUrl":"https://doi.org/10.1007/s12288-022-01588-0","url":null,"abstract":"<p><strong>Purpose: </strong>Hemophilia B is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor IX (FIX) clotting activity. This study evaluated safety and efficacy of nonacog alfa, a recombinant human blood coagulation FIX replacement product, in males aged 12-65 years with hemophilia B (FIX activity ≤ 2%) with or without inhibitors in India.</p><p><strong>Methods: </strong>In this multicenter, open-label, post-approval phase 4 study, participants were treated for up to 8 weeks, with up to a 4-week screening period and a subsequent post-treatment 28-day safety observation period. Intravenous nonacog alfa 40 IU/kg (range 13-78 IU/kg) was administered at intervals of 3-4 days, in accordance with the approved local product document.</p><p><strong>Results: </strong>A total of 25 participants were enrolled and completed the study. No participants developed FIX inhibitors during the study, experienced treatment-related adverse events (AEs) or serious AEs, or developed a thrombotic event and/or hypersensitivity reaction. No participants experienced bleeding events requiring on-demand treatment with nonacog alfa. Seventeen bleeding episodes (16 spontaneous and 1 traumatic) were reported in 10 participants; all occurred post treatment, with the exception of a minor gum-bleeding event, and were managed without treatment. The mean (SD) annualized total factor consumption (TFC) per patient was 224,582 (75,527) IU; the mean (SD) annualized TFC by weight per patient was 3639 (573) IU/kg.</p><p><strong>Conclusion: </strong>Nonacog alfa was safe and effective for the prevention of hemorrhagic episodes in Indian males with congenital, severe hemophilia B. No participants developed FIX inhibitors, and no new safety signals were reported.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542435/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41166417","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}