Indian Journal of Hematology and Blood Transfusion最新文献

{"title":"Applicability of Hscore and Adapted HLH 2004 for Diagnosis of Adult HLH in Indian population-A Prospective Study.","authors":"Megha Verma, Nitin Gupta, Jasmita Dass, Vandana Arya, Deepika Gupta, Amrita Saraf, Sabina Langer, Jyoti Kotwal, Ajay Sharma","doi":"10.1007/s12288-024-01902-y","DOIUrl":"10.1007/s12288-024-01902-y","url":null,"abstract":"<p><strong>Introduction: </strong>Hemophagocytic Lymphohistiocytosis (HLH) is a severe and rapidly progressive immune disorder that can be life threatening if not diagnosed and treated promptly. The HLH2004 guidelines, originally devised for the diagnosis of HLH in pediatric subjects, lack due utility in the adult population. Despite the development of the H-score and adapted HLH 2004 criteria, a critical gap remains in their validation and optimization for diverse populations, including the Indian adult population.</p><p><strong>Methods: </strong>In this prospective observational study, clinical data of 120 patients with suspected HLH were collected. The utility of the H-score and the adapted HLH 2004 for the diagnosis of HLH in adults was studied with respect to expert consensus. The best cut-off value of the calculated H-score for the diagnosis of HLH in our set up was studied using Receiver Operating Characteristic (ROC) curve analysis.</p><p><strong>Results: </strong>Of 120 adult inpatients suspected of having HLH, 66 were diagnosed with HLH, 37 did not have HLH, and 17 had undetermined status based on expert consensus. Infections were the most common underlying cause, present in 60% of the cases, followed by malignancies (15%). The adapted HLH 2004 criteria demonstrated higher sensitivity (90.9%) but lower specificity (86.5%) compared to the H-Score at the standard cut-off of 169, which had a sensitivity of 81.82% and specificity of 94.59%. ROC curve analysis identified an optimal H-score cut-off of 145, improving the sensitivity to 92.4% and specificity to 89.2%.</p><p><strong>Conclusion: </strong>Our study highlights the importance of region-specific diagnostic criteria for HLH in adults, particularly in settings where infection is prevalent. By optimizing the H-score cut-off to 145, we improved the diagnostic sensitivity and specificity in our cohort. These findings support the need for tailored diagnostic tools to enhance HLH detection in diverse populations.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"622-628"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676210","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":"Plasminogen Activator Inhibitor 1 in Patients with Primary Immune Thrombocytopenia: A Case Control Study.","authors":"Mahmoud Souliman Sheeba, Menna Allah Zakaria Abou Elwafa, Rana G Abdelfatah","doi":"10.1007/s12288-024-01873-0","DOIUrl":"10.1007/s12288-024-01873-0","url":null,"abstract":"<p><p>Primary immune thrombocytopenia (ITP) is a condition associated with decreased platelet count plus subsequent high risk of bleeding. Many studies mentioned that ITP also has a higher risk of developing thrombosis, which may have a significant influence on the disease management. Plasminogen activator inhibitor-1 (PAI-1), a fibrinolysis inhibitor, is implicated within the procoagulant state of ITP patients. To assess the PAI-1 serum level in ITP patients, non-immunologic thrombocytopenia controls (TC) and healthy controls (HC), and to correlate PAI-1 level with clinical variables. Serum PAI-1 was measured by ELISA in 45 ITP patients, 22 TC and 22 HC. All participants were subjected to complete clinical assessment and examination. Bleeding severity was assessed using SMOG bleeding score. Levels of PAI- 1 were discovered to be significantly higher in ITP patients compared to TC and HC with a high significant difference between the three groups with <i>p</i> value < 0.0001. Only three ITP patients developed thrombosis. PAI-1 level showed no significant relation with platelet count, SMOG bleeding score, thrombosis, treatment with thrombopoietin agonists (TPO RA) and splenectomy. Primary ITP patients showed significantly higher PAI-1 level compared to non-immunologic thrombocytopenic patients and healthy controls. This finding may help in understanding the suggested increased thromboembolic risk in ITP patients. However, no association with bleeding score and risk of thrombosis was found in this study.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"599-604"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676283","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":"Evaluation of Optical Platelet Counts in HORIBA Yumizen H2500 and Platelet Counts by Digital Morphology Platform in Cases of Thrombocytopenia with Platelet Interference Flag: Finding Solutions with Automation in High Volume Laboratory.","authors":"Gautam Gopal Bhagwat, Pushkar Admane, Ankur Lath, Prithu Das, Shreya Mallick, Pankti M Patel","doi":"10.1007/s12288-024-01889-6","DOIUrl":"10.1007/s12288-024-01889-6","url":null,"abstract":"<p><p>Platelet counts which are generated by impedance methods (PLT-I) in cases of thrombocytopenia can be unreliable due to interference of red cells. We evaluated utility of optical platelet counts on HORIBA Yumizen H2500 and platelet count by digital morphology platform CellaVision DM1200 with multivariate analysis for understanding causes in cases of thrombocytopenia with platelet interference flag. A total of 150 samples with platelet counts < 150 X 10⁹/L (PLT-I) were selected for this study. Platelet counts were then analyzed in parallel within 6 h of collection using optical technique (PLT-O) and also PLT-D (Digitalized Smear techniques). Platelet count on peripheral smear was also performed by two pathologists (MPC). Statistical analysis using Pearson's correlation, Bland-Altmann plot analysis and multivariate regression analysis was performed. The mean platelet count with PLT-I was 74.6 × 10⁹/L while this was significantly higher in PLT-O (108.9 × 10⁹/L). Pearson's correlation between PLT-O and MPC as well as PLT-D and MPC showed a good correlation between the two values, proving the utility of PLT-O over PLT-I in cases of RBC -platelet interference flags. Multivariate regression analysis demonstrated a positive effect of MPV and indicated that with increase in MPV the difference between PLT-I and PLT-O is significantly affected. PLT-O was found to give more accurate platelet count than PLT-I in cases of thrombocytopenia with platelet interference flag. Also, digital morphology is a useful alternative to manual platelet count estimation.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"592-598"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267744/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676297","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":"Efficiency of PET-CT in Reducing the Usage of Radiotherapy in Childhood Hodgkin Lymphoma: A Single Center Experience.","authors":"Goran Milosevic, Rodic Predrag, Nada Krstovski, Dejan Skoric, Alhayek Nabil, Milos Veljkovic, Jelena Lazić","doi":"10.1007/s12288-024-01878-9","DOIUrl":"https://doi.org/10.1007/s12288-024-01878-9","url":null,"abstract":"<p><p>Hodgkin Lymphoma is a complex malignancy with unique features, primarily affecting children and young adults. The disease's sensitivity to radiation therapy and the young age of onset underscore the importance of optimizing treatment strategies to minimize both acute and long-term toxicities associated with radiotherapy. In light of these considerations, our study aimed to evaluate whether [18 F]FDG-PET/CT assessment at interim and end-of-treatment timings, in comparison to conventional CT scans, led to a decrease or increase in unnecessary patient exposure to radiotherapy. The study involved 61 pediatric patients diagnosed and treated for Hodgkin lymphoma at our institution between 2009 and 2022. Patients were categorized into two groups based on treatment protocols: Group 1 received conventional CT imaging protocols, while Group 2 received [18 F]FDG-PET/CT-based protocols. The results demonstrated that [18 F]FDG-PET/CT-based protocols led to a reduction in the frequency of radiotherapy compared to conventional CT imaging (32% vs. 52%). This statistically significant difference highlights the potential benefits of [18 F]FDG-PET/CT in guiding treatment decisions and reducing unnecessary radiation exposure. Our research re-emphasize the potential of [18 F]FDG-PET/CT as a valuable tool in the management of pediatric patients with Hodgkin lymphoma in terms of more precise diagnosis and reduction of unnecessary treatment and toxicities.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"504-510"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267784/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676221","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":"Inotuzumab Ozogamicin in Indian Patients with B-Cell Acute Lymphoblastic Leukemia.","authors":"Pronamee Borah, Vivek Mohan, Sachin Jain, Varun Capoor, Rahul Naithani","doi":"10.1007/s12288-024-01881-0","DOIUrl":"https://doi.org/10.1007/s12288-024-01881-0","url":null,"abstract":"<p><p>There is scarcity of data on utility of inotuzumab ozogamicin (InO) in relapsed refractory acute lymphoblastic leukemia in India. This is a retrospective study. Twelve patients (3 children) with a median age of 22.5 years (8-80 Years) were evaluated. Two patients received upfront InO therapy due to poor general condition. Four patients received IO due to persistent MRD positivity. Six patients had active disease of which 5 were multiply relapsed. Six patients received single-agent InO. Median number of cycles were 2 (1-6). All received inotuzumab on scheduled days and there was no treatment delay due to cytopenia. Ten patients (83%) achieved measurable residual disease (MRD) negative complete remission (CR). Seven of these ten patients became MRD-negative after 1 cycle only. All MRD-positive patients converted to MRD-negative remission. Four patients underwent allogeneic bone marrow transplant. Two patients continued standard maintenance therapy. Six patients succumbed. The median follow-up of the entire cohort is 10.7 months (2-32 months). Five out of twelve (41.6%) patients are alive and in MRD-negative remission at a median follow-up of 22 months (11-32 months). Inotuzumab ozogamicin is an effective agent for inducing deep remissions in patients with B-cell acute lymphoblastic leukemia.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"665-669"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676303","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":"NPM1 Measurable Residual Disease: A Narrative Review.","authors":"Yadav Chitresh, Nathany Shrinidhi, Kumar Nikhil M, Swaminathan Anusha, Panda Rastogi Neha, Verma Kanika, Saini Manish, Verma Hari Stuti, Danewa Arun, Dua Vikas, Bhargava Rahul","doi":"10.1007/s12288-025-01958-4","DOIUrl":"https://doi.org/10.1007/s12288-025-01958-4","url":null,"abstract":"<p><p>Acute myeloid leukemia (AML) with mutated <i>NPM1</i> (Nucleophosmin 1) is a distinct entity in the 5th edition of the World Health Organization (WHO) Classification as well as a favourable risk category in the 2022 European LeukemiaNet (ELN) Recommendations for AML. The development of myriad techniques for detection of <i>NPM1</i> Measurable Residual Disease (MRD) has led to improved patient outcomes; however, there is no one winner. Each modality has its own nuances and caveats, and hence using a synoptic and integrated approach is recommended to avoid potential false negatives. Therapy guided by genomic MRD especially in decision for timing of transplant has been reported in literature, which has shown benefits of regular longitudinal monitoring of <i>NPM1</i>. This is a narrative review which discusses in detail the testing modalities for <i>NPM1</i> MRD in AML. Keywords like 'NPM1\", \"MRD\", \"NGS\", were searched in Pubmed, Embase and Scopus and a total of 40 articles were included. Since this is not a systematic review or meta-analysis no inclusion or exclusion criteria were laid and no formal statistics was done. This article was constructed in pursuit of raising awareness for this unmet need, as well as to amalgamate all old and new knowledge in this space. The future is artificial intelligence and machine learning which may pave the way for risk scores.</p><p><strong>Supplementary information: </strong>The online version contains supplementary material available at 10.1007/s12288-025-01958-4.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"453-459"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267802/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676307","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":"Ablation of CD38 Alleviates the Secondary Aggregation of Platelets.","authors":"Mazhar Mushtaq, Uh-Hyun Kim","doi":"10.1007/s12288-024-01875-y","DOIUrl":"10.1007/s12288-024-01875-y","url":null,"abstract":"<p><p>CD38 is an ectoenzyme that plays an essential role in mobilizing intracellular Ca²⁺. Here, we intend to demonstrate the role of CD38 in platelet primary and secondary aggregation and suggesting the need to inhibit primary aggregation for cardiovascular patients. Mouse platelets were used in this study. Platelet aggregation, in vitro thrombus formation, release reactions, and calcium signalling experiments were performed in response to thrombin, a normal agonist of platelets. For aggregation, pathway-specific inhibitors were used to differentiate between primary and secondary aggregation. In an in vitro setting, the formation of a thrombus revealed a distinctive pattern on the collagen-coated surface when comparing two types of platelets. Platelets positive for CD38 exhibited smaller yet more aggregated platelets than CD38-negative platelets. Moreover, in vitro, the aggregation process exhibited distinct patterns for the two types of platelets. By employing various inhibitors, we were able to distinguish between the primary and secondary aggregation pathways, both upstream and downstream. The release reaction and calcium signalling were valuable for identifying primary and secondary aggregation events based on their respective time frames. Distinct variations in thrombus formation and aggregation patterns suggest the involvement of CD38. The significant difference in the second minute of calcium signalling and release reaction evidently established a distinct time interphase between primary and secondary aggregation.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"585-591"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676278","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":"Spur Cell Anemia: Wolf in Disguise!","authors":"Akanksha Garg, Tejas Modi, Ripal Goswami, Loma Parikh, Nidhi Jain","doi":"10.1007/s12288-024-01908-6","DOIUrl":"10.1007/s12288-024-01908-6","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"751"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267788/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676290","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":"Chediak Higashi Syndrome Under the Microscope.","authors":"Tharageswari Srinivasan, Abhishek Daroach, Narender Kumar, Abarna Thangaraj, Rakesh Kumar Pilania","doi":"10.1007/s12288-024-01892-x","DOIUrl":"https://doi.org/10.1007/s12288-024-01892-x","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"745-746"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267804/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676219","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":"Alpha-Thalassemia Caused by αα^{IVSI-1(AGGT> AGAT)} (HBA1: c.95 + 1G > A) Mutation and its Combinations with Other Forms of Thalassemia or Hemoglobinopathy in Northern Thailand.","authors":"Sakorn Pornprasert, Chedtapak Ruengdit, Manoo Punyamung, Orapan Sripichai","doi":"10.1007/s12288-024-01895-8","DOIUrl":"https://doi.org/10.1007/s12288-024-01895-8","url":null,"abstract":"<p><p>The αα^{IVSI-1(AGGT> AGAT)} mutation is typically disregarded during routine thalassemia testing because of its mild or absent pathology in heterozygous individuals as well as its interactions with modulators that can result in variable phenotypes, particularly in β-thalassemia (β-thal) and hemoglobin E (HbE) carriers. This study was performed to analyze the αα^{IVSI-1(AGGT> AGAT)} mutation in Northern Thailand using targeted next-generation sequencing (NGS). Hb analysis was performed by HPLC and/or CE methods. The α-thal-1 --^SEA, --^Thai, and --^{Chiang Rai} type deletions were detected by real-time PCR with high-resolution melting analysis, and the α-thal-2 (-α^3.7 and -α^4.2) deletion was detected by conventional gap-PCR. Thalassemia genotypes were further investigated using an NGS panel targeting the coding regions of the <i>HBA1</i>, <i>HBA2</i>, and <i>HBB</i> genes. The αα^{IVSI-1(AGGT> AGAT)} mutation was misdiagnosed by routine thalassemia diagnostic methods. NGS results showed that 7 (3 Burmese and 4 Thai originations) of 1107 (0.63%) blood samples were heterozygous for the αα^{IVSI-1(AGGT> AGAT)} mutation, which appeared in 5 different genotypes. Clinical and hematological features varied with their combination forms. The targeted NGS analysis utilized in this study is a promising genetic testing method for the identification of uncommon globin gene mutations. It shows particular promise in thalassemia screening and genetic counseling.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":"41 3","pages":"674-679"},"PeriodicalIF":0.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267114/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676280","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}