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The diagnosis of Central Nervous System tuberculosis (CNS TB) had been always an enigma as yield of Mycobacterium tuberculosis in cerebrospinal fluid (CSF) is very low and diagnosis mainly rested on combination of clinical, CSF analysis and radiological findings. We attempted to find whether 16s rRNA primer based real time Polymerase chain reaction (PCR) aids in diagnosis of tubercular meningitis (TBM) and tuberculoma. Literature is flooded with newer techniques for the diagnosis of neuro-tuberculosis by real time PCR. But no clear cut guidelines are available about their role. 40 cases of tubercular meningitis, diagnosed on basis of Modified Ahuja Criteria and 40 controls were included in this prospective study. All patients were evaluated clinically and investigated in detail including cerebrospinal fluid analysis. Acid Fast Bacilli staining, culture for TB by Bac T Alert 3-D system, 16s rRNA real time PCR assay, chest X ray, computed tomography scans of head, contact survey using chest X-ray, Mantoux test and hemogram. Comparative analysis of real time PCR were done in control group vs. cases of TBM diagnosed on the basis of Modified Ahuja criteria. Results: CSF for tuberculosis by real time PCR was positive in 26/40 cases (65%) of TBM, as against 2/40 controls. Keeping the CSF culture as the gold standard its sensitivity is 62.50 (95% confidence intervals- 54.7, 85.2), specificity is 77.40 (95% confidence intervals- 54.7, 85.2), positive predictive value is 46.15 and negative predictive value is 88.89. Modified Ahuja criteria can serve as useful tool in diagnosis. CSF analysis by real time PCR has shown better sensitivity and quicker results than culture. However, it cannot be recommended alone as it was negative in about a third of cases. But its significance is underlined in early diagnosis and as an adjunct to culture. [ABSTRACT FROM AUTHOR]

Analysis of pediatric tetanus cases was carried out to study the predictors of mortality and role of tetanus immunoglobulin (TIG). Shorter incubation period, onset time and autonomic dysfunction were significantly associated with mortality and may be used to stratify patients requiring intensive care. TIG may not have independent role in decreasing mortality in sick patients. [ABSTRACT FROM AUTHOR]

We report a 5-year-old girl who presented with acute onset paraparesis with differential loss of sensation. Magnetic resonance imaging of spine revealed exophytic intramedullary mass lesion from T12 to L1. Peroperatively, the diagnosis was confirmed as abscess. The patient recovered following decompression and antibiotic treatment. [ABSTRACT FROM AUTHOR]

The article describes a case study of an eight-year-old girl with transfusion-dependent anemia diagnosed with congenital sideroblastic anemia due to a mutation in the SLC25A38 gene, leading to defective heme synthesis and ring sideroblast formation. Topics include the clinical features and genetic basis of congenital sideroblastic anemia, the significance of SLC25A38 mutations, and differential diagnosis involving bone marrow findings such as vacuolations.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN characterized by reciprocal translocation in the ABL1 and BCR region of chromosomes 9 and 22 respectively. Progression to the blast phase in chronic myeloid leukemia results in a poorer prognosis. It can be of either myeloid, lymphoid or a mixed lineage. Progression to the promyelocytic blast phase is very rare, and there are no evidence-based guidelines for its management. Thrombosis in CML is not well defined. Thrombosis can be seen in patients with acute promyelocytic leukemia (APL) with venous thrombosis (VTE) being more common than arterial thrombosis. Ischemic stroke as the presenting feature of blast phase progression in CML is extremely rare. We report a case of CML who presented to us with acute ischemic stroke and subsequently was diagnosed as CML transformed to the promyelocytic blast phase. She was successfully treated with dasatinib along with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO). [ABSTRACT FROM AUTHOR]

Background & objectives: Glanzmann thrombasthenia (GT) is a rare, inherited autosomal recessive disorder characterized by qualitative or quantitative deficiency of integrin αIIbβ3 [glycoprotein IIb (GPIIb)/IIIa, CD41/CD61] diagnosed by absent or reduced platelet aggregation to physiological agonists, namely, collagen, adenosine-di-phosphate, epinephrine and arachidonic acid. The objective of this study was to quantitate platelet surface GPs, classify GT patients and relate the results with the severity of bleeding and platelet aggregation studies. Methods: Fifty one patients of GT diagnosed by platelet aggregation studies were evaluated for the expression of CD41, CD61, CD42a and CD42b on platelet surface by flow cytometry. The association between the clinical phenotype based on bleeding score and GT subtype on flow cytometric evaluation was assessed. Results: Twenty four (47%) patients of GT were classified as type I (as CD41/CD61 were virtually absent, <5%), six (11.8%) patients as type II (5-20% CD41/CD61) and 21 (41.2%) as type III or GT variants as they had near normal levels of CD41 and CD61. Type III GT patients had significantly lower numbers of severe bleeders (P=0.034), but the severity of bleeding did not vary significantly in type I and II GT patients. In all GT patients, mean CD41 expression was found to be lower than mean CD61 expression (P=0.002). Interpretation & conclusions: Type I GT was found most common in our patients and with lowered mean CD41 expression in comparison with CD61. Type III GT patients had significantly lower numbers of severe bleeders, but the severity of bleeding did not vary significantly in type I and II GT patients. [ABSTRACT FROM AUTHOR]

Introduction: Current molecular research has shown the several oncogenic pathways that give rise to the peripheral T-cell lymphoma, not otherwise defined (PTCL, NOS) subtypes, which alter prognosis and might have predictive value. This study was conducted to assess the immunohistochemistry (IHC) algorithm by Amador et al for the subtyping of PTCL, NOS and determine its applicability in relation to the clinicopathological profile., Methods: This study included 43 patients with PTCL, NOS diagnosis. Following the use of IHC for the transcription factors GATA3, TBX21, CCR4, and CXCR3, two pathologists subtyped the samples. Comprehensive clinicopathological correlation was carried out., Results: Applying the algorithm of Amador et al., cases were classified into GATA3 (20), TBX21 (15), and unclassified (8) subtypes. No significant association with clinical parameters of subtypes or CD4/ CD8 positivity was observed. Although a higher proportion of cases in the TBX21 subgroup showed a polymorphic population compared with the GATA3 subgroup, which had a monomorphic population, no significant p-value (0.111) was observed. Two Lennert lymphomas were classified into the GATA3 subgroup. Multivariate analysis showed no significant difference in overall survival (p-value = 0.105) and progression-free survival (p-value = 0.0509) between IHC-defined subtypes; trends indicate that overall survival and progression-free survival are worse in the GATA3 subgroup., Conclusion: Although the algorithm is reproducible, a proportion of cases remains unclassifiable and may require additional investigation and gene expression profiling. The GATA3 subgroup was found to have a monomorphic population with a poor overall prognosis and thus requires a larger sample size for validation., (© 2024 John Wiley & Sons Ltd.)

Background: Mesenchymal stem cells (MSCs) have emerged as promising candidates for immune modulation in various diseases that are associated with dysregulated immune responses like Graft-versus-Host-Disease (GVHD). MSCs are pleiotropic and the fate of MSCs following administration is a major determinant of their therapeutic efficacy., Methods: Human MSCs were derived from bone marrow (BM) and Wharton's Jelly (WJ) and preconditioned through exposure to hypoxia and induction of apoptosis, either sequentially or simultaneously. The immune programming potential of preconditioned MSCs was evaluated by assessing their effects on T cell proliferation, induction of Tregs, programming of effector T-cell towards Th2 phenotype, macrophage polarization in the direct co-culture of MSCs and aGVHD patients-derived PBMNCs. Additionally, efferocytosis of MSCs and relative change in the expression of immunomodulatory soluble factors were examined., Results: Our study demonstrated that hypoxia preconditioned apoptotic MSCs (BM-MSCs, WJ-MSCs) bear more immune programming ability in a cellular model of acute Graft-versus-Host-Disease (aGVHD). Our findings revealed that WJ-MSCs HYP+APO were superior to BM-MSCs HYP+APO for immune regulation. These induced the differentiation of CD4 + T-cell into Tregs, enhanced Th2 effector responses, and simultaneously mitigated Th1- and Th17 responses. Additionally, this approach led to the polarization of M1 macrophages toward an M2 phenotype., Conclusion: Our study highlights the potential of WJ-MSCs conditioned with hypoxia and apoptosis concurrently, as a promising therapeutic strategy for aGVHD. It underscores the importance of considering MSC apoptosis in optimizing MSCs-based cellular therapy protocols for enhanced therapeutic efficacy in aGvHD., (© 2024. The Author(s).)

Purpose: To determine the rate of platelet transfusion in patients with cirrhosis and severe thrombocytopenia (platelet counts <50 × 10 9 /L) undergoing high-risk invasive procedures when prescribed by thromboelastography (TEG) compared with empirical and on-demand transfusion strategies., Materials and Methods: This was a single-center, single-blinded, randomized controlled trial. Patients with cirrhosis and severe thrombocytopenia undergoing high-risk invasive procedures were randomized into 3 groups: TEG group, transfusions based on TEG parameters; standard of care (SOC) group, 3 units of random donor platelets before procedure; and on-demand group, transfusions based on procedural adverse events/clinician's discretion. The primary outcome was periprocedural platelet transfusion in each arm., Results: Eighty-seven patients were randomized (29 in each group) with no significant differences in demographics/coagulation profile/procedures. The median platelet count was 33 × 10 9 /L (interquartile range, 26-43 × 10 9 /L). Percutaneous liver biopsy was the most common procedure (46, 52.9%). Significantly lower number of patients in the TEG group received platelets (4 cases, 13.8%; 95% CI, 3.9-31.7) compared with SOC group (100%; 95% CI, 88.1-100; P < .001). Four patients in the on-demand group received platelets (13.8%; 95% CI, 3.9-31.7). Minor (World Health Organization [WHO] Grade 2) procedure-related bleeding occurred in 3 (10%; 95% CI, 2.2-27.4) patients in the TEG-guided transfusion group compared with 1 (3.4%; 95% CI, 0.1-17.8) patient each in the SOC and on-demand groups (P = .43), although the study was not powered for comparison of bleeding rates. No bleeding-related mortality was observed in any of the 3 groups., Conclusions: TEG-prescribed transfusion reduced prophylactic transfusions in patients with cirrhosis and severe thrombocytopenia undergoing high-risk invasive procedures. The study was not powered for comparison of bleeding rates., (Copyright © 2024 SIR. Published by Elsevier Inc. All rights reserved.)

Objectives: Deficiency of adenosine deaminase-2 (DADA2) is a monogenic disorder closely resembling polyarteritis nodosa (PAN) and can present to physicians across various specialties. Through this case series, we aim to describe the clinical spectrum and outcome of Indian children with DADA2. We aimed to study the clinical spectrum and outcome of Indian children with DADA-2., Methods: The deidentified data from all participating centres were entered in an excel sheet, and the coordinating centre (All India Institute of Medical Sciences, New Delhi) screened the data for accuracy and completeness., Results: We enrolled 16 children (11 females) in the study; the mean (SD) age at the time of onset of symptoms for males and females was 46.2 (47) and 73.6 (50.4) months, respectively. The most common clinical feature in this cohort was fever and rash in 80% of patients. More than half of children n, (%) [8, (53%)] had a CNS stroke. The other clinical features were hypertension [5(33%)], anaemia [3 (20%)] and arthralgia/arthritis in 4 (26%). These children were managed with various immunomodulators: steroids [13, (86%)], anti-TNF agents [(12, (80%)], cyclophosphamide [2 (13%)] and mycophenolate mofetil [3 (20%)]. The median (IQR) duration of follow-up for this cohort was 17 (10, 29) months. Fourteen children achieved remission and none had recurrent strokes after the initiation of anti-TNF drugs., Conclusion: DADA-2 closely resembles PAN; early age of onset and CNS stroke are striking differentiating features from classic PAN. Most children respond well to anti-TNF agents without serious adverse events during short-term follow-up., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

To evaluate the utility of CD43 and CD200 in differentiating chronic lymphocytic leukemia (CLL) from other mature B-cell neoplasms. This was a cross-sectional study on patients diagnosed with B-cell neoplasms on flowcytometry. The median fluorescence intensity (MFI) of CD43, CD200 expressing neoplastic B-cells were compared between the CLL and non-CLL B-cell neoplasms followed by receiver operating characreristic curve (ROC) analysis. In addition, the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of CD43 and CD200 in diagnosing CLL were analysed. A total of 137 patients were included. The CLL group consisted 87 patients and non-CLL group consisted 50 patients. The Mann-Whitney U test showed significant CD43 expression (U = 997.5, Z= - 5.265, p  < 0.001) and CD200 expression (U = 932.0, Z = - 5.5, p  < 0.01) in CLL patients compared to non-CLL patients. The area under the curve were 0.771 and 0.786 for MFI of CD43 and CD200 in differentiating CLL from non-CLL group respectively. The optimal cut-off of MFI for CD43 and CD200 were 1323 and 1775 respectively. The sensitivity, specificity, PPV and NPV of CD43 in diagnosing CLL cases were 97.7%, 66%, 83.3% and 94.2% respectively. The sensitivity, specificity, PPV and NPV of CD200 in diagnosing CLL cases were 100%, 32%, 71.9% and 100% respectively. CD43 and CD200 are useful markers in differentiating CLL from other mature B-cell neoplasms with higher MFI expression of both markers found in CLL., Competing Interests: Conflict of interestNo conflict of interest., (© The Author(s), under exclusive licence to Indian Society of Hematology and Blood Transfusion 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Competing Interests: Competing interests: The authors declare no conflict of Interest.

During the last two decades the world has seen an increase in the use of Hematopoietic Stem Cell Transplant (HSCT) which has led to its worldwide expansion. Since, HSCT unit is an advanced set up, developing and maintaining a successful hematopoietic stem cell transplant program with a properly functioning unit enhances the credibility of any tertiary level medical facility especially for a country like ours which is in its early expanding phase of providing transplant services. The underlying principle for designing any HSCT facility is to maintain the highest possible level of aseptic environment for patients undergoing the transplant in order to prevent healthcare associated infections. Basic premises of designing the entire HSCT unit was to ensure restricted access to the facility and having an aseptic environment by implementing infection control parameters in design elements, which are explained subsequently in the article. The present manuscript describes the project experience of creating a positive pressure isolation facility for HSCT patients at a tertiary care hospital, India, which is a resource limited setting with an emphasis on need assessment, key elements in planning and designing along with the challenges associated with it., Competing Interests: Conflict of interestThe authors have no conflicts of interest to declare that are relevant to the content of this article., (© The Author(s), under exclusive licence to Indian Society of Hematology and Blood Transfusion 2023.)

Background: Multiple myeloma remains an incurable disease, with the majority of patients relapsing after autologous stem cell transplant (ASCT). After relapse, second transplant remains one of the therapeutic options, along with novel agents., Methods: We reviewed the data of our patients who underwent ASCT for myeloma (N = 202) over the last two decades (2004-2019). Of these, 12 patients underwent a second transplant., Results: Out of 12 patients, nine underwent second autologous stem cell transplant, whereas three received an allogeneic stem cell transplantation (Allo-SCT). Median progression-free survival (PFS) after the first ASCT was 32 months (5-84 months). Median interval between both the transplants was 35 months (4-159 months). Median age of our cohort which underwent second transplant was 56 years. Overall response rate (ORR) post-second transplant on day +100 was 83.3%, without any transplant-related mortality (TRM). With the use of preemptive plerixafor, none of our patients required a second day for stem cell harvest. Median CD34 dose of stem cells infused was 4.11 × 10 6 /kg. Similar to the first ASCT, the median time to neutrophil and platelet engraftment was 11 and 12 days, respectively. At a median follow-up of 41 months, estimated 3-year PFS and overall survival (OS) was 37% ± 15% and 63% ± 15%, respectively., Conclusion: ">Among all relapsed myeloma patients who were transplant eligible, 11% underwent a second transplant. Second transplant is well tolerated with similar time to engraftment after first ASCT. Hence, we believe that second transplant is a feasible, cost-effective option in a resource-limited setting, which should be more widely utilized.

Primary splenic lymphomas are rare with the majority of lymphomas in spleen being secondary to an extra-splenic lymphoma. We aimed to analyze the epidemiological profile of the splenic lymphoma and review the literature. This was a retrospective study including all splenectomies and splenic biopsies from 2015 to September 2021. All the cases were retrieved from Department of Pathology. Detailed histopathological, clinical and demographic evaluation was done. All the lymphomas were classified according to WHO 2016 classification. A total of 714 splenectomies were performed for a variety of benign causes, as part of tumor resections and for the diagnosis of lymphoma. Few core biopsies were also included. A total of 33 lymphomas diagnosed in the spleen, primary splenic lymphomas constituted 84.84% (n = 28) of the cohort with 5 (15.15%) having the primary site elsewhere. The primary splenic lymphomas constituted 0.28% of all the lymphomas arising at various sites. Adult population (19-65 years) formed the bulk (78.78%) with a slight male preponderance. Splenic marginal zone lymphomas (n = 15, 45.45%) comprised of major proportion of cases followed by primary splenic diffuse large B-cell lymphoma (n = 4, 12.12%). Splenectomy was the main course of treatment for SMZL with a good overall outcome, with chemotherapy ± radiotherapy forming the mainstay in other lymphomas. Lymphomas in spleen can be infiltrative or a primary, hence proper clinic-radiological and pathological evaluation is required. Appropriate management is guided by the precise and detailed evaluation by the pathologist, requiring understanding of the same., Competing Interests: Conflict of interestThere is no conflict of interest amongst the authors., (© The Author(s), under exclusive licence to Indian Society of Hematology and Blood Transfusion 2022. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

A man in his early 30s presented to us with progressive shortness of breath limiting activities of daily living. An important clue in history was the episode of a deep vein thrombosis 5 years ago treated with short-term anticoagulation. His echocardiography revealed elevated estimated pulmonary artery systolic pressure. A CT pulmonary angiography confirmed chronic thromboembolic pulmonary hypertension. Blood investigations established primary antiphospholipid syndrome. He underwent pulmonary endarterectomy, relieving his symptoms and was started on indefinite oral anticoagulation with warfarin. He is currently under follow-up with no recurrence of thrombosis.We wish to highlight the importance of an appropriate workup of venous thrombosis in all patients. Antiphospholipid syndrome is a rare disease with important implications in the management of patients with thromboses. The delay in his diagnosis had several causes including the unclear distinction between provoked and unprovoked thrombosis and socioeconomic factors in a developing nation limiting referral and testing., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Magnetic resonance image (MRI) brain tumor segmentation is crucial and important in the medical field, which can help in diagnosis and prognosis, overall growth predictions, Tumor density measures, and care plans needed for patients. The difficulty in segmenting brain Tumors is primarily because of the wide range of structures, shapes, frequency, position, and visual appeal of Tumors, like intensity, contrast, and visual variation. With recent advancements in Deep Neural Networks (DNN) for image classification tasks, intelligent medical image segmentation is an exciting direction for Brain Tumor research. DNN requires a lot of time & processing capabilities to train because of only some gradient diffusion difficulty and its complication., Methods: To overcome the gradient issue of DNN, this research work provides an efficient method for brain Tumor segmentation based on the Improved Residual Network (ResNet). Existing ResNet can be improved by maintaining the details of all the available connection links or by improving projection shortcuts. These details are fed to later phases, due to which improved ResNet achieves higher precision and can speed up the learning process., Results: The proposed improved Resnet address all three main components of existing ResNet: the flow of information through the network layers, the residual building block, and the projection shortcut. This approach minimizes computational costs and speeds up the process., Conclusion: An experimental analysis of the BRATS 2020 MRI sample data reveals that the proposed methodology achieves competitive performance over the traditional methods like CNN and Fully Convolution Neural Network (FCN) in more than 10% improved accuracy, recall, and f-measure., (© 2023. The Author(s).)

We report the case of a woman in her early 20s with a history of recurrent infection, atopic dermatitis, filariasis and bilateral purulent ear discharge since childhood with tonsillar enlargement on examination. She was started on supportive care and evaluated for primary immunodeficiency disease. Blood investigations revealed increased IgM levels with reduced IgG, IgA and IgE levels. Radiological imaging of the chest revealed bilateral bronchiectasis. Otoscopic examination showed features suggestive of chronic suppurative otitis media. Next-generation sequencing identified homozygous single base pair deletion in exon 2 of the activation-induced cytidine deaminase gene. Thus, a diagnosis of hyper-IgM syndrome type 2 was confirmed. The patient was started on monthly intravenous immunoglobulin replacement therapy and is currently symptomatically better, and she remains under regular follow-up., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

The study was designed to review the demographic, clinical, and pathologic characteristics of follicular helper T cells (TFH)-derived nodal PTCL in India including angioimmunoblastic T-cell lymphoma (AITL), peripheral T-cell lymphoma (PTCL) with follicular helper T cell phenotype (P-TFH), and follicular T-cell lymphoma with additional immunohistochemistry (IHC) and RHOAG17V mutational analysis, as well as their impact on survival. This retrospective study included 88 cases of PTCL that were reclassified using IHC for TFH markers (PD1, ICOS, BCL6, and CD10) and dendritic-meshwork markers (CD21, CD23). Cases of TFH cell origin were evaluated for RHOAG17V mutation using Sanger sequencing and amplification-refractory mutation system-polymerase chain reaction (PCR) (validated using cloning and quantitative PCR) with detailed clinicopathologic correlation. Extensive re-evaluation with added IHC panel resulted in a total of 19 cases being reclassified, and the final subtypes were AITL (37 cases, 42%), PTCL-not otherwise specified (44, 50%), P-TFH (6, 7%), and follicular T-cell lymphoma (1, 1%). The presence of at least 2 TFH markers (>20% immunopositivity) determined the TFH origin. AITL patients tended to be male and showed increased presence of B-symptoms and hepatosplenomegaly. Histomorphology revealed that 92% of AITL cases had pattern 3 involvement. Sanger sequencing with conventional PCR did not yield any mutation, while RHOAG17V was detected by amplification-refractory mutation system-PCR in AITL (51%, P =0.027) and P-TFH (17%), which was validated with cloning followed by sequencing. Cases of RHOAG17V-mutant AITL had a worse Eastern Cooperative Oncology Group performance status initially but fared better in terms of overall outcome ( P =0.029). Although not specific for AITL, RHOAG17V mutation shows an association with diagnosis and requires sensitive methods for detection due to low-tumor burden. The mutant status of AITL could have prognostic implications and translational relevance., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Acute promyelocytic leukemia (APL) is a medical emergency. The diagnosis of APL requires morphological examination, cytochemistry, immunophenotyping, and reverse transcriptase polymerase chain reaction (RT-PCR) for PML::RARA or its variants. However, due to the rapid development of complications, diagnosis often relies on morphology and cytochemistry for early treatment. Herein, we describe a 72-year-old gentleman who presented with pancytopenia diagnosed as acute promyelocytic leukemia with an unusual morphology. The bone marrow smear showed 80% myelocyte-like cells with prominent granules and maturation arrest, with an occasional neutrophil. On careful re-examination of the peripheral smear and bone marrow, an occasional poorly preserved cell with a bundle of Auer rods was identified. Cytochemistry for MPO was strongly positive in abnormal promyelocytes and flow cytometry showed positivity for MPO, CD13, CD33, and CD117 and was negative for CD34 and HLA-DR. Cytogenetics showed a complex karyotype of 45,XY, -14, t(15;17)(q24;21)t(14;21)(q11.2;p13)[10]/ 45, XY, idem, add(5)(q35)[5]/ 45,X,-Y[5]. RT-PCR for PML-RARA was positive for the bcr-3 transcript and FISH was positive for t(15;17) (q24;q21). The take home point from our case is to look for the presence of cells with bundle of Auer rods whenever there is pancytopenia with the presence of myelocyte-like cells with prominent granulations.

To clarify the significance of bone marrow fibrosis and amyloid deposition in plasma cell neoplasm, a retrospective cross-sectional study for a period of 3 years was conducted. Patients who underwent bone marrow aspiration and biopsy with suspicion of plasma cell neoplasms were included in the study. The bone marrow findings were correlated with clinical profile of the patient along with biochemical parameters, cytogenetics, Fluorescent in situ hybridization (FISH) wherever available. A total of 273 bone marrow aspirates and biopsies of patients with suspected plasma cell neoplasms were analyzed. There were 181 male patients and 92 female patients (Male: Female = 1.96: 1). There were 245 cases of multiple myeloma (89.7%), 8 cases of primary amyloidosis (2.9%) and 6 monoclonal gammopathy of undetermined significance (MGUS) (2.1%), 5 cases of plasmacytoma (1.8%) and 4 cases of smouldering myeloma (1.4%), 5 cases of POEMS syndrome (1.8%). Bone marrow fibrosis was noted in 12 patients at diagnosis (4.3%). Among the parameters studied, only the mean Hemoglobin was significantly low in patients with marrow fibrosis. Amyloid deposition in various organs including bone marrow, kidney, liver etc., were noted in 17 patients overall (6.2%). In conclusion, the incidence of fibrosis (4.3%) and amyloidosis (6.2%) associated with plasma cell neoplasms were much lower in our study as compared to published studies.

Introduction: Cancer-associated thrombosis is a leading cause of morbidity and mortality in malignancy patients. Prophylactic anticoagulation is under-utilized and the cost of low-molecular-weight heparin (LMWH) and direct oral anticoagulants is a major barrier in developing countries., Material and Methods: A retrospective analysis was performed of all cancer-associated thrombosis patients attending the thrombosis clinic at a tertiary-level referral hospital based in North India between 2011 and 2015. Patient demographics and disease-related parameters were collected and analyzed., Results: A total of 771 patients attended the thrombosis clinic during study period, of which 64 cases were malignancy-associated. Of these, 56% of the patients were female and 20% were bedridden. The median age was 48.5 years, adenocarcinoma (48%) being the most common histological subtype. Gynecological malignancies (30%) were the most common malignancies, followed by genitourinary (11%) malignancies. Most of the cases occurred during first year of diagnosis (51%), and only 14% occurred after 3 years. Most of the patients were on combined treatment. Almost 40% of the patients developed thrombosis within 30 days of surgical treatment. Lower limb thrombosis was the most commonly seen type (56%), while abdominal and pulmonary thrombosis were both seen in 5%. Patients were managed with LMWH and vitamin K antagonists (84.3%) and only 6.25% with LMWH alone. Direct oral anticoagulants were not commonly used during the study period., Discussion: At the hospital studied, most of the cases occurred early in the disease course. Postoperative prophylaxis could have contributed towards reducing thrombosis in the peri-operative period. Early suspicion and prompt treatment can improve quality of life in such patients., Competing Interests: Conflicts of interest None., (Copyright © 2021. Published by Elsevier España, S.L.U.)

Background: Treatment of acute promyelocytic leukaemia has emerged as a major success in hemato-oncology. While literature from the developed world boasts of outstanding outcomes, there is a paucity of data from the developing world. This study aimed to assess complications and outcomes of acute promyelocytic leukaemia in a resource-constrained setting., Methods: We retrospectively collected data from patients diagnosed with APL from January 2016 to December 2020., Results: Sixty-four patients were treated-32 in both the Sanz high and low-risk groups. In the Sanz low-risk group, 12.5% of patients received ATRA with daunorubicin and 81.25% received ATRA with ATO. In the Sanz high-risk group, 18.8% of patients received ATRA with daunorubicin, 34.3% received ATRA with daunorubicin and ATO while 40.6% received ATRA with ATO. 56.25% of patients developed differentiation syndrome. The incidence was higher in Sanz high-risk group as compared to Sanz low-risk group. 57.4% of patients had an infection at the time of presentation. 62.5% of patients developed neutropenic fever during treatment. 17.2% of patients developed pseudotumor cerebri. The 4-year EFS and OS were 71.25 and 73.13%, respectively. Sanz low-risk group had a better 4-year EFS and OS as compared to the Sanz high-risk group. Haemoglobin at presentation and Sanz high-risk group were associated with poorer outcomes with a hazard ratio of 0.8 and 3.1, respectively. Outcomes in high-risk patients were better with the use of ATRA + ATO + daunorubicin., Conclusion: In the Indian population, APL patients have a high incidence of differentiation syndrome, pseudotumor cerebri, and infections during induction. CR, EFS, and OS compared to the developed world can be achieved with optimal therapy. Low haemoglobin at presentation and Sanz high-risk group were associated with poorer outcomes. ATRA, ATO, and daunorubicin combination is the preferred protocol for treating high-risk patients.

Background: Coronavirus disease 2019 (COVID-19) causes abnormalities in the hemostatic system, collectively known as COVID-associated coagulopathy. The dynamics of clot formation are best discerned by whole-blood viscoelastic tests, such as thromboelastography (TEG). We aimed to assess the various abnormalities seen on TEG and explored the predictors of outcomes in these patients., Methods: Thromboelastography was performed for 28 patients with COVID-19 using an automated thromboelastogram. The hemostatic condition was categorized as hypercoagulable in 17 (63%), hypocoagulable in 2 (7%), and normal in 8 (30%) based on TEG variables, such as reaction time , time until clot reaches a fixed strength, alpha angle, maximum amplitude, and clotting index. Laboratory parameters and clinical outcomes were compared between hypercoagulable and normal groups., Results: Twenty-seven patients with a median age of 50 years (interquartile range, 40-60 years), male-to-female ratio of 0.9:1, median C-reactive protein of 25.7 (10.9-108.8) mg/L, serum ferritin of 693 (317-1031) µg/L, and albumin 2.9 (2.6-3.3) g/dL were included. The median prothrombin time/international normalized ratio and activated partial thromboplastin time were within normal range in the hypercoagulable and normal groups. The severity of COVID-19 was mild in 6 (22.2%), moderate in 2 (7.4%), and severe in 19 (70.4%) patients. Twenty-eight-day mortality among patients with hypocoagulable and hypercoagulable states was higher than normal coagulation status. (log-rank test, P  = .002)., Conclusions: Hypercoagulable state, together with a severe inflammatory state, is common in patients with COVID-19, despite thromboprophylaxis. TEG assesses coagulation status better than conventional coagulation tests. Coagulation abnormalities are associated with poor outcomes., (© 2022 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH).)

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia with or without bleeding. Mild to moderate thrombocytopenia can be seen with thyrotoxicosis and hypothyroidism. This study was conducted to assess prevalence of thyroid dysfunction in ITP and impact on treatment and outcomes. This prospective study included patients more than 12 years old, diagnosed as ITP. Serum Thyroxine (T4) and Thyroid Stimulating Hormone levels and anti-thyroid peroxidase (TPO) antibodies were done by electro- chemiluminescence immunoassay. A total of 168 patients were enrolled, with thyroid function tests available in 146 patients. Mean age was 30.6 years, with 67.8% females (n = 114). Sixty percent patients had chronic ITP and 25% had persistent ITP. Overall prevalence of thyroid disease was 25.7% in the study population, with overt hypothyroidism seen in 21 (16.4%) patients, subclinical hypothyroidism in 9 (7.0%) patients and subclinical thyrotoxicosis in 3 (2.3%) patients, while no patient had overt thyrotoxicosis. Thyroid status of the patients did not correlate with duration of ITP or response to treatment. Presence of anti TPO antibodies was associated with abnormal thyroid function and chronic ITP. The prevalence of thyroid dysfunction is increased in ITP patients. Many such cases have anti TPO antibodies, suggesting autoimmune pathology. Role of glucocorticoids, estrogens and systemic illness in these findings must be further studied before firm conclusion can be drawn about their routine inclusion in diagnostic work up., (© Indian Society of Hematology and Blood Transfusion 2021.)

In COVID 19 pandemic, delivery and access of health care services have become challenging. Telemedicine services can be considered for management of patients with hematological diseases. This study included all patients who enrolled for telemedicine facility for hematology from May 15 to July 15, 2020. Patient's demographic and disease related parameters were recorded during the teleconsultation call. Overall satisfaction of attending doctor and patients were also recorded. A total of 1187 teleconsultation appointments were taken, of which 944 (79.6%) were successfully attended. Median age of patients was 38 years (range- 0.5-78 years), with 38% females. 55% of successful calls were from patients suffering a malignant hematological disorder. 24% had an active complaint pertaining to their disease or treatment. Of these, 162 (17%) were asked for a physical consultation. A significant association was found between the requirement of physical consultation and diagnosis ( p  < 0.001), absence of active complaint (< 0.0001) and education level of responder ( p  = 0.008). Patients understand that teleconsultation is helpful in preventing COVID-19 infection (71.4%) and avoids outpatient department rush (14.5%) associated with physical appointments; and around 80% patients were satisfied with the teleconsult. With the emergence of COVID 19, many localities under partial lockdown with constant fear of contacting virus amongst patients and health care providers, we can clearly see the advantages as well as feasibility of telemedicine services for our patients. The acute surge in telemedicine could be harnessed in the future to provide comprehensive and integrated care to patients of hematological disorders., (© Indian Society of Hematology and Blood Transfusion 2020.)

The Coronavirus disease-19 (COVID-19) pandemic has in multiple ways affected healthcare delivery to non-COVID patients throughout the world. Adequate transfusion services are fundamental in ongoing therapy of patients with hematological ailments. We present the transfusion services in the hematology daycare under the department of Hematology and supported by the Blood Bank at our institution for the period 12th April 2020-30th June 2020, which saw the stringent lockdown and unlocking Phase I in India, declared in lieu of the pandemic. A 56 % reduction in total transfusion sessions was observed in 2020 (588 sessions given to 176 patients) compared to 1336 sessions in 516 patients over the same period in 2019. The reductions were seen across the different blood components (packed red blood cells [PRBC]: 585 vs. 1840, platelet rich plasma: 372 vs. 1313, single donor platelet 18 vs. 16), with a significant reduction in the mean PRBC transfused per PRBC transfusion session (1.11 vs 1.99, p<0.001) in 2020, compared to 2019. There were however no major differences in the transfusion practices across the different phases of the lockdown. Our study highlights the detrimental reduction in transfusion services due to the COVID-19 pandemic and related lockdown and showcases the remedial strategies taken to maximize transfusion support to patients during this period. Our observations might help to provide insights to adequately combat possible similar adverse situations in the future., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

COVID-19 has emerged as a pandemic with lung being the primarily afflicted organ. Deranged hemostasis has been observed in patients with COVID-19 with scales tipped towards a prothrombotic state. The pathogenesis differs from disseminated intravascular coagulation with a primary pulmonary localization. This is referred to as pulmonary intravascular coagulopathy with strong component of thrombo-inflammation. This is reflected in the lab tests with an increase in D-dimer which correlates with severity and outcomes of disease. Common coagulation tests such as prothrombin time, activated partial thromboplastin time are only mildly prolonged while most patients have normal to increased fibrinogen and marginal thrombocytopenia. Overall, the patients have an increase in venous and arterial thrombotic events especially in ICU patients. Routine thromboprophylaxis with low molecular weight heparin is recommended in all hospitalized patients to reduce the incidence of thrombosis. Bleeding is uncommon and treated with blood products transfusion. This review shall discuss the hemostatic abnormalities in COVID-19 patients and their impact on prognosis. In addition, strategy of thromboprophylaxis and various academic society guidelines are discussed in detail., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interests., (© Indian Society of Hematology and Blood Transfusion 2020.)

Plasma cell leukemia (PCL) is an aggressive rare leukemic variant of multiple myeloma (MM). We aim to present 4 years data on clinical profile and treatment outcomes of Primary PCL (PPCL) patients treated at tertiary care cancer centre from Northern India. To analyse response and safety profile of a PPCL with or without stem cell transplantation. Retrospectively reviewed and analysed PPCL patient's data at our centre from January-2013 to June-2017. Total 11 PPCL patients diagnosed among 240 MM patients during study period. Eight were males. Only 10 patients were started on treatment. Four (n = 4/10) patients underwent stem cell transplantation. Overall response rate was 70% (n = 7). Eleven culture positive bacterial infections (bloodstream = 2, urinary tract = 3; pulmonary = 6) were recorded. Four patients had fungal infections. One patient had Herpes Zoster infection. Relapse rate of entire cohort was 50% (n = 5). Median PFS and OS of entire cohort was 11 months (95% confidence interval 6.3-15.6) and 21 months (95% C.I. 1-49.8) respectively. The estimated PFS and OS at 1 year of transplanted versus nontransplanted patients were 71% + 24% versus 0% ( P  = 0.96) and 71% + 24% versus 15% + 19% ( P  = 0.234) respectively. Treatment with PIs + IMAs followed by transplants (single/double) might improve depth and duration of remission and OS. Patients should be treated with indefinite maintenance therapy to control disease., Competing Interests: Conflict of interestAll authors have declare that they have no conflict of interest., (© Indian Society of Hematology and Blood Transfusion 2019.)