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Objectives: Eβ-thalassemia, the most serious form of HbE syndromes, may develop pre-diabetes (PD) and diabetes mellitus (DM), together constituting abnormal glucose homeostasis (AGH) as an endocrinopathy. This study aims to assess AGH prevalence and pathogenesis in this thalassemia subtype. Material and Methods: A cross-sectional study was conducted at a tertiary care hospital from February 2017 to December 2018 (1.9 years). One hundred and four HbEβ-thalassemia patients were randomly selected aged ≥5 years, irrespective of transfusion requirement. AGH was diagnosed by the American Diabetes Association criteria. The patient’s history, relevant examination details, and parameters related to glucose homeostasis were studied. The homeostasis assessment (HOMA) model of Oxford University was used, and formulae were applied to calculate HOMA-insulin resistance (IR) or HOMA-β (β-cell function). Results: The status of glucose homeostasis was as follows: Normal glucose homeostasis tolerance 83/104(79.8%), PD 20/104(19.2%), and DM one(1%). The patient’s age, age of starting transfusions, and HOMA-IR were significantly related to AGH. AGH was inversely associated with the age of starting chelation, though not significant (P = 0.07). There was no statistical significance of AGH development, with transfusion dependence (P = 0.63), family history of DM (P = 0.42), hepatitis C (P = 0.36), and higher ferritin levels (800/1000/1500/1700 ng/ml) (P > 0.5)/HOMA-β (P > 0.5). Conclusion: HbEβ-thalassemia patients are prone to develop AGH including overt diabetes. It is related to the patient’s age, age of initiation, and duration of transfusion therapy. The likely mechanism of pathogenesis is IR, though pancreatic β-cell destruction may also be contributory.

Objectives: Treatment for children with newly diagnosed immune thrombocytopenia (ITP) includes corticosteroids, out of which prednisolone is commonly used. High-dose dexamethasone (HD-DXM) treatment for children with newly diagnosed ITP can produce better outcomes than prednisone, as stated by many studies. A few articles compare HD-DXM and prednisolone as frontline therapies in newly diagnosed adult ITP but data on pediatric ITP comparing these two drugs are scarce. Material and Methods: It was a randomized, prospective, and cohort study. After enrolment, checking the inclusion and exclusion criteria, each child was randomly distributed into two groups; one cohort was treated with HD-DXM in the dose of 40 mg/m2 (maximum daily dose 40 mg) for 4 days once every 28 days for three cycles; and one with prednisolone in the dose of 2 mg/kg/day (maximum 60 mg/day) for 2 weeks, with quick tapering. A total of 42 children were enrolled, 21 in each cohort. Results: The overall response (OR) was 42.9% with HD-DXM therapy. The OR and median time to response were similar in both HD-DXM and prednisolone cohorts. There was also no significant difference in incidences of adverse effects between the two cohorts. Conclusion: Thus, this study confirms that HD-DXM is a safe, effective, and non-inferior option compared to prednisolone as frontline therapy in newly diagnosed ITP in children.

The 3'-untranslated region (3'-UTR) is well known to be associated with the post-transcriptional regulation, because of the presence of important sequences that influence the fate of mRNA, and thus, in protein synthesis. The present study describes a point mutation on the β-globin 3'-UTR, +1506 (AC) (

Introduction Acute leukemia (AL) is among the most common treatable cancers in childhood but many children are forced to abandon therapy. Objective We have explored reasons for treatment abandonment through this study. Materials and Methods Retrospective analysis from an ongoing registry data of childhood AL patients, where all newly diagnosed AL patients of ≤18 years of age (June 2014–November 2017) were included. Patients >18 years of age, having any history of treatment of AL outside our institute, and/or patients with relapsed AL were excluded. The primary outcome of the study was treatment abandonment rate. Results A total of 710 AL patients were included in the study, average distance traversed to reach the hospital being 161.66 km. Most children were aged 1 to 10 years (49.4%, n = 351), followed by >10 to 18 years (46.6%, n = 331), and ≤1 year age (3.9%, n = 28). The commonest symptoms were fever (67.4%), pallor (38.6%), bleeding (11.5%), bone pain (13.8%), neck swellings (14.9%), and, rarely, testicular swellings or Superior vena cava (SVC) syndrome (1.1%). A high abandonment rate was noted prior to leukemia subtyping (35.2%, n = 250) mostly among males 62% (n = 155) and the 1- to 10-year group 55.6% (n = 139). A total of 460 (64.8%) patients were subsequently subcategorized by immunophenotyping. Precursor B-cell (Pre-B) Acute Lymphoblastic Leukemia (ALL) in 307 (43.2%) patients was the commonest subtype, followed by early Pre-B ALL (Pro-B ALL) in 10 (1.4%), T-cell ALL (T-ALL) in 51 (7.1%), Acute Myeloid Leukemia (AML) in 45 (6.3%), Acute Promyelocytic Leukemia (APML) in 28 (3.9%), and Mixed Phenotypic Acute Leukemia (MPAL) in 19 (2.6%). Conclusion The most common group of patients was aged 1 to 10 years (median age: 5 years). An abandonment rate of 35.2% was seen prior to complete diagnostic workup. The reason for this high abandonment, despite good disease prognosis, is a relevant social and health issue, and needs further evaluation. The problems discussed in this study are relevant to lower-income families and areas where health care is not easily accessible. The government agencies, nongovernment organizations and society would need to work together to overcome these issues.

Background Haemophilia has been associated with increased prevalence of low bone mineral density (BMD) which in turn may aggravate haemophilic arthropathy. Dual-energy X-ray absorptiometry (DEXA) is the gold standard for assessing BMD but is not widely available across India. Markers of bone turnover like bone-specific alkaline phosphatase (b-ALP) reflect osteoblastic turnover and may be surrogate to low BMD. Aim To evaluate how bone health in people with haemophilia (PWH) can be assessed by serum vitamin D3 and b-ALP level, correlated with the degree of arthropathy. Methods In this cross-sectional study, people with haemophilia A and B of all severities with arthropathy involving ≥3 joints were included. The number of joints affected by haemophilic arthropathy was recorded. Hemophilia Joint Health Score (HJHS) and Pettersson score were calculated for each patient. Levels of serum calcium, phosphorus, vitamin D3 and b-ALP were assayed in all cases. Results A total of 320 PWH were included; the majority (85%; 272/320) had severe haemophilia, 13.44% (43/320) moderate haemophilia and 1.56% (5/320) mild haemophilia. With increasing age, the number of joints involved increased significantly (r=0.2250, p Conclusion PWH with severe disease and haemophilic arthropathy have higher than normal b-ALP, which in turn reflects increased bone turn over and low BMD. Hence, b-ALP may be a useful marker to help assess bone health in PWH, particularly in settings where access to DEXA scans is constrained.

Evaluation of bone marrow infiltration in lymphoma is usually done by bone marrow biopsy (BMB). This study analyzed the utility ofTreatment-naïve lymphoma patients underwent bothThe study population consisted of 80 patients and comprised 37 Hodgkin's lymphoma (HL) patients, 30 aggressive non-HL (NHL) and 13 indolent NHL patients. The majority of the aggressive NHLs were diffuse large B-cell lymphoma (20/30) and major indolent lymphoma was follicular lymphoma (5/13). When compared to BMB, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of focal (±diffuse) marrow FDG uptake on

The study was aimed to evaluate the performance of a newly developed non-invasive and non-contact bilirubin measurement device (AJO-Neo) as an alternative to the conventional invasive biochemical method of total serum bilirubin (TSB) estimation in preterm and term neonates suffering from hyperbilirubinemia associated with risk factors, and/or undergoing phototherapy. The safety and efficacy of the device were assessed in 1968 neonates with gestational ages ranging from 28 to 41 weeks and suffering from incidences of hyperbilirubinemia. Linear regression analysis showed a good correlation between AJO-Neo and the conventional method of TSB (Pearson’s coefficient, r = 0.79). The small bias (0.27 mg/dL) and limits of agreements (− 3.44 to 3.99 mg/dL) were within the range of clinical acceptance. The device was also precise in the measurement of bilirubin levels in all subgroups of the study. The receiver operator curve (ROC), that takes account of both sensitivity and specificity of a device showed high efficacy of the device (area under the curve, AUC = 0.83) in the detection of bilirubin. While monitoring the bilirubin level during phototherapy, the device indicated promising results showing good agreement with TSB. Specificities and sensitivities of the device indicated a much higher accuracy in neonates with associated risk factors for hyperbilirubinemia. Hence, the newly developed device (AJO-Neo) is reliable in measuring bilirubin level in preterm, and term neonates irrespective of gestational or postnatal age, sex, risk factors, feeding behavior or skin color.

Waldenström macroglobulinemia (WM) is an indolent B-cell neoplasm in which lymphoplasmacytic cells are accumulated in the bone marrow. This rare disease is incurable with the available treatments and its management is generally based on risk-adapted methods. We present here six cases of WM with their clinicopathological profile and response to therapy. This is a retrospective observational study conducted in a tertiary referral center in Eastern India from October 2014 to December 2016. The clinical manifestations, diagnostic challenges, and response to therapy for WM were analyzed. A total of six cases of WM were analyzed, of which five were male; the mean age was 65 years. The most common presentation was transfusion-dependent anemia, followed by hyperviscosity. All the patients had bone marrow infiltration by lymphoplasmacytoid cells, and serum protein electrophoresis and immunofixation showed immunoglobulin M kappa monoclonal protein. Bortezomib, dexamethasone, and rituximab (BDR) was the most commonly used regimen. Complete and very good partial responses were seen in one patient (16.7%) each and partial response in 3 (50%) patients. There was disease progression to diffuse large B-cell lymphoma in one patient (16.7%). After a 26-month follow-up, four of six patients (66.67%) remained free of disease progression. A very low incidence of WM was reported, and patients had a varied clinicopathological spectrum. A combination of BDR is a promising frontline therapy in the patients of WM.

To investigate the effectiveness of low dose secondary/tertiary prophylaxis in severe Hemophilia A children and determine improvements in their daily life. Thirty Hemophilia A children (≤ 12 y) with factor VIII

Hb E-β thalassemia is the most common form of hemoglobinopathy in Southeast Asia and eastern India. Iron overload resulting from blood transfusion and increased intestinal iron absorption promotes the formation of reactive oxygen species (ROS), leading to oxidative stress, organ dysfunction, and tissue damage. Of these, cardiovascular complications are the leading cause of mortality. Impaired endothelial function is a biomarker of vascular health in patients with cardiovascular risks. Therefore, assessment of endothelial function is a useful prognostic tool. In the present study, 60 E- β thalassemia patients and 60 healthy, age, sex matched control subjects were taken. The mean hemoglobin and ferritin of thalassemic patients were 7.43gm/dl and 1032 mcg/dl respectively. The vascular health was compared by measuring flow-mediated vasodialation (FMD), arterial elastic parameters, and carotid intima-medial thickness (CIMT). There was lower FMD (7.49%) and higher CIMT (0.46mm) in thalassemic group than control (10.52 % and 0.36mm respectively) (p valuelt; 0.05). Also arterial stiffness is elevated and arterial distensibility is lower in thalassemic patients than control. Among the thalassemic patients FMD or CIMT did not correlate with serum ferritin value. So, the E- β thalassemia patients had poor vascular health and are at a higher risk of developing atherosclerosis and cardio-vascular complication than normal population. The vascular dysfunction does not correlate with serum ferritin value, so regular monitoring with Doppler study is required for early diagnosis of subclinical atherosclerosis in this group of patients. However the effects of chelation therapy, Hydroxyurea, or other targeted therapies needs to be validated by further study.

Purpose Immune thrombocytopenia (ITP) is primarily considered a bleeding disorder; its impact on patients’ health-related quality of life (HRQoL) is under-recognized. We aimed to assess how aligned patient and physician perceptions are regarding ITP-associated symptoms, HRQoL, and disease management in India. Methods Patients and physicians (hematologists/hemato-oncologists) from India who participated in the global ITP World Impact Survey (I-WISh) were included in this subgroup analysis (survey). Physicians were recruited via a local, third party recruiter in India. In addition to completing a survey themselves, physicians were asked to invite consulting patients on a consecutive basis to complete a survey. All surveys were completely independently by the respondents online in English. The respondents took 30 min to complete the questionnaire. Patients also completed the newly developed ITP Life Quality Index (ILQI) that included 10 questions on the impact of ITP on the following: work or studies, time taken off work or education, ability to concentrate, social life, sex life, energy levels, ability to undertake daily tasks, ability to provide support, hobbies, and capacity to exercise. Results A total of 65 patients and 21 physicians were included in this study. Average disease duration from diagnosis-to-survey-completion was 5.3 years. The most severe symptoms reported by patients at diagnosis were menorrhagia (15 of 19 patients [79%]), anxiety surrounding unstable platelet counts (17 of 28 patients [61%]), and fatigue (27 of 46 patients [59%]); these were also the key symptoms they wanted to be resolved. In contrast, physicians perceived petechiae (19 of 21 patients [90%]), bleeding-from-gums (8 of 21 patients [86%]), and purpura (16 of 21 patients [76%]) as the most common symptoms. While the important treatment goals for patients were healthy blood counts (42 of 65 patients [65%]), improved QoL (35 of 65 patients [54%]), and prevention of worsening of ITP (33 of 65 patients [51%]), physicians’ goals were reduction in spontaneous bleeding (17 of 21 physicians [81%]), better QoL (14 of 21 physicians [67%]), and symptom improvement (9 of 21 physicians [43%]). More than half the patients reported that ITP affected their work life/studies, social life, and energy levels, thereby negatively impacting their QoL. Patients were almost entirely dependent on family and friends for support. Conclusions This survey highlights the substantial discrepancy in patients’ and physicians’ perceptions regarding ITP-associated symptoms and treatment goals in India. Based on the identified gaps, educating physicians on aspects of ITP beyond bleeding, and highlighting patients’ under-recognized symptoms/needs through support-systems should be prioritized in the future.

Careful screening of bilirubin level in newborns is mandatory as per American Academy of Pediatrics (2004), to reduce incidents of kernicterus and acute bilirubin encephalopathy. Although, invasive capillary collection of blood and subsequent biochemical test is considered a gold standard for jaundice detection in neonates, transcutaneous bilirubin measurement using various non-invasive instruments is also used sporadically across the globe. The major aim of this study was to develop a non-invasive spectrometry-based technique for measurement of neonatal bilirubin level as an alternative of total serum bilirubin (TSB) test without limitations of other available bilirubinometers.The instrument comprises of a light source and a spectroscopic detector. A light beam from source incident on the neonatal nail plate through optical fibers. The retro reflected light is acquired using the detector. An indigenously developed software is used to acquire and analyze the optical signal and to calculate the bilirubin value. The instrument was calibrated and validated in reference to TSB on 1033 subjects.The result (r = 0.95 and P0.001) indicates a strong correlation between the bilirubin value obtained from our method and TSB. Time variant analysis of the subjects undergoing phototherapy provided a good correlation (r = 0.98). The repeatability test result shows the mean coefficient of variation is less than 5.0%.The indigenously developed non-invasive technique successfully detects the bilirubin level in newborns under various physiological conditions with high accuracy and precision.

The current study was conducted to assess response to immunosuppressive therapy (IST) in acquired aplastic anaemia (AA). It was a retrospective and prospective observational study. Patients were diagnosed as per standard international guidelines and IST was started as per standard protocol. Patients were followed up at 3 months and 6 months for assessment of response as per published standard guidelines. Total 76 cases were included in the study. The median age of the study population was 36 years with a range of 6-66 years with a male to female ratio of 2.04:1. Most common clinical presentation was pallor followed by bleeding. Commonest type of disease in the study group was severe AA. Among total 76 patients, 32 patients received Atgam and 44 patients received Thymogam. Within 3 months of ATG administration, 4 patients died and 1 patient was lost to follow up. At 3 months, 2 (2.63%) patients were on complete response (CR), 32 (42.10%) patients were in partial response (PR) and 37 (48.68%) patients were on no response (NR). Overall response (OR) at 3 months was 44.73%. At 6 months 5 (6.57%) patients were in CR, 43 (56.57%) patients in PR and 23 (30.26%) patients in NR; the OR was 63.14%. Overall response at 3 months was 44.73% and overall response at 6 months was 63.14%. The study revealed better overall survival for patients with ATGAM treatment than THYMOGAM treatment arm.

OBJECTIVE: To compare the blood transfusion pattern between children with transfusion dependent Eβ-thalassemia and β-thalassemia major. METHODS: 168 children (age 3 months to 12 years) with transfusion dependent Eβ thalassemia and β thalassemia major were admitted to the hospital. 120 children who met our inclusion criteria, were selected and detailed history including various parameters indicating the blood transfusion pattern were recorded. RESULTS: In this study 72 children (60%) of the patients were transfusion dependent Eβ thalassemia. They started receiving blood transfusion (BT) at a later age(p

Background Hemophilia A is an X chromosome-linked bleeding disorder caused by the deficiency of coagulation factor VIII (FVIII). The majority of the Indian population with hemophilia A use plasma-derived clotting factors and, in some instances, fresh frozen plasma and cryoprecipitate. Safer and more efficient treatment options are needed for this group of patients. Objectives To assess the safety of turoctocog alfa, a third-generation recombinant FVIII molecule, for the treatment and prophylaxis of bleeding episodes in previously treated Indian patients with moderate or severe hemophilia A. Patients/methods This single-country, multicenter, open-label, nonrandomized trial enrolled 60 patients who received prophylactic treatment with turoctocog alfa for 8 weeks, which corresponded to a minimum of 20 exposure days. Confirmed development of FVIII inhibitors during the 8-week treatment period was evaluated. Other assessments included frequencies of adverse drug reactions (ARs), serious adverse reactions, drug-related allergic reactions, and infusion reactions during the 12-week period after the first treatment; hemostatic effect of turoctocog alfa for the treatment of bleeding episodes; and total annualized dose of turoctocog alfa administered during the 8-week treatment period. Results No incidence of FVIII inhibitors was detected. No safety concerns such as ARs, serious ARs, or drug-related allergic reactions were noted. The hemostatic success rate for the treatment of bleeding episodes with turoctocog alfa was 81.6%. Conclusions The trial results demonstrated that turoctocog alfa is a safe treatment option for the prophylaxis and treatment of bleeding episodes in previously treated adolescent and adult patients with hemophilia A in the Indian population.

Humanity has three great enemies: fever, famine, and war (Bryan 1996) and fever by far is the greatest and the most terrible of the three. Fever is the defining event of severe infection and defining fever has been a major challenge with different proposed thresholds. The mean oral temperature in health is 98.2 °F, with a range of 96 °F–100.8 °F and a slight diurnal variation (Mackowiak et al. 1992). Fever, as defined by the Society of Critical Care Medicine and the Infectious Disease Society of America, is an oral temperature of above 101 °F (O’Grady et al. 1998). Fever in a neutropenic patient has different defining thresholds. Febrile neutropenia is defined by a single oral temperature of 101 °F or a temperature of more than 100.4 °F sustained over a period of 1 h. Axillary temperature may not reflect core body temperature and rectal temperature measurement may result in colonizing of gut commensal into the surrounding mucosa, and hence both are discouraged (Freifeld et al. 2011). The defining temperature threshold at times rests at the intensivist’s discretion and the clinical condition of the patient.

Objective of this paper: Early detection of anemia (low hemoglobin count) at point-of-care (PoC) in a low resource setting is challenging given the associated capital and recurring costs of the device. In this paper, we have developed a faster and reliable method to screen the hemoglobin level in the whole blood in a resource limited point of care setting. We have also investigated a simple scattering-based technique to predict the overall morphology of the red blood cells. Methods: In this paper, we have developed and validated a digital camera-based spectrometer for the early detection of anemia with the whole blood of 10- $\mu \text{L}$ volume in a low resource PoC setting. The developed device consists of two LEDs [Green (570 nm) and Red (631 nm)], a web camera, a fluid sensing chamber (cuvette holder), and associate electronics. Validation of Beer–Lambert law using the blood of various degree of dilution has been achieved in this in vitro experiment. Major results: A significant number of human subjects (300) having a wide range of hemoglobin counts in a hospital is tested and found to reproduce results from a gold standard automated hematology analyzer. Our developed microcuvette requires only 10- $\mu \text{L}$ unprocessed whole blood sample for the device. Conclusions: The device is expected to serve as a minimally invasive e-health care device for the anemia screening in any resource-limited point of care setting.

Chronic myeloid leukemia is a stem cell disease with the presence of Philadelphia chromosome generated through reciprocal translocation of chromosome 9 and 22. The use of first- and second-generation tyrosine kinase inhibitors has been successful to an extent. However, resistance against such drugs is an emerging problem. Apart from several drug-resistant mechanisms, drug influx/efflux ratio appears to be one of the key determinants of therapeutic outcomes. In addition, intracellular accumulation of drug critically depends on cell membrane fluidity and lipid raft dynamics. Previously, we reported two novel cell-penetrating peptides (CPPs), namely, cationic IR15 and anionic SR11 present in tryptic digest of Abrus agglutinin. Here, the potential of IR15 and SR11 to influence intracellular concentration of imatinib has been evaluated. Fluorescent correlation spectroscopy and lifetime imaging were employed to map membrane fluidity and lipid raft distribution following peptide-drug co-administration. Results show that IR15 and SR11 are the two CPPs which can modulate membrane fluidity and lipid raft distribution in K562 cells. Both IR15 and SR11 significantly reduce the viability of CML cells in the presence of imatinib by increasing the intracellular accumulation of the drug.

To compare the efficacy and safety of imatinib and cytarabine (ara-c) combination versus imatinib monotherapy in newly diagnosed patients with chronic phase chronic myeloid leukemia (CML-CP).This prospective, randomized study included adult patients (age18 years) with newly diagnosed CML-CP. Patients received either a single oral dose of imatinib 400 mg/day in combination with a subcutaneous injection of ara-c 20 mg/mOf 30 patients included, 14 were randomized to imatinib + ara-c and 16 to imatinib alone. Complete hematologic response (CHR) at 3 months was higher with imatinib + ara-c vs. imatinib alone (100% vs. 87.5%, P = 0.48). The median time to achieve CHR was significantly (P0.001) lower with imatinib + ara-c (32.07 vs. 23.43 days). Molecular response at 3 months was significantly higher (P = 0.04) with imatinib + ara-c vs. imatinib alone (100% vs. 68.75%). Complete cytogenetic response was also higher with imatinib + ara-c vs. imatinib alone (42.85% vs. 25% at 6 months and 71.4% vs. 62.5% at 12 months). Neutropenia followed by thrombocytopenia and anemia were the most common AEs. Grade 3/4 hematological and nausea events were significantly (P0.05) higher with imatinib + ara-c. Other nonhematological events were not significantly different between the treatments. The median follow-up duration was 20 months (range: 15-23 months).Imatinib with low-dose ara-c can be considered as a potential first-line treatment option for CML-CP.

Anemia in India carries a major disease burden. This includes both nutritional anemias, of which iron deficiency anemia (IDA) is the commonest and inherited hemolytic anemias like β thalassemias (β-TT). In Eastern and North-Eastern India about 25–40% of the public blood bank blood is consumed by patients with β Thalassemia. Moreover, 51% of Indian women in the reproductive age suffer from IDA. Most vulnerable group of anemic patients is in the rural underserved regions. This underserved population can only be served through the use of artificial intelligence (AI), automation, supported by telemedicine. To combat the problems of both IDA and β-thalassemia by early diagnosis at the point-of-care– we have developed Suśruta - an Artificial Intelligence (AI) driven robust smartphone-based health care application. This App uses five major components of AI; namely: (1) Natural language processing (NLP) to analyze the unstructured clinical data and translate it into computer understandable 3rd Generation SNOMED and ICD10 ontologies; (2) Speech Synthesis; (3) Artificial Neural Network (ANN) with Machine Learning and Deep Learning (ML/DL) on 60,283 labelled common blood counts (CBC) and High Performance Liquid Chromatography (HPLC) data collected over 8 years by a teaching hospital in Kolkata for β-TT screening; (4) Computer Vision and Image Processing techniques to interpret hemoglobin content in blood through non-invasive analysis of conjunctiva and nailbed images; and (5) NoSQL and Big-data Graph database-driven Bayesian Knowledge Network for Evidence Based Medicine and Bayesian Outcome Tracing for Predictive Medicine. Unlike previous systems, the ML/DL technique of β-Thalassemia carrier screening with CBC improved the accuracy of screening by two folds compared similar approaches analyzing CBC with Mentzer Index. Moreover, the uniqueness of Suśruta is that the App is robust and works both in an offline and online mode at resource constrained point-of-care. This is the first time AI is used for comprehensive anemia care by early diagnosis, which empowers the ordinary health workers in rural underserved communities. Furthermore, it will introduce the concept of patient empowerment and person centered care by changing the definition of point-of-care in rural India.

Hemoglobin (Hb)-F inducers are known to improve Hb level and transfusion dependence in thalassemia. This pilot study was conducted to assess the efficacy and safety of Hb-F inducer thalidomide compared to hydroxyurea (HU) in Hb E-β thalassemia patients.This was a prospective interventional single-centre study with 45 Hb E-beta thalassemia patients equally divided into group-I (thalidomide+folic acid), group-II (HU + folic acid) and group-III (folic acid). Response was assessed at various time intervals with 12-months follow up period. Primary end points were increment in Hb, Hb-F level and improvement in transfusion requirement; secondary end point were tolerability and safety.There was 100% responder (R: Hb-increment ≥1 g/dl) in group-I with 66.67% major responder (MaR: Hb-increment ≥2 g/dl), while there were 40% and 0% responder in group-II and III respectively. Hb-increment was significantly (p-value0.0001) better in thalidomide arm compared to HU. The Hb-increment was attributable to both increase in Hb-F levels and reduction in ineffective erythropoiesis in thalidomide arm. Transfusion reduction was significantly better in group-I compared to group-II (100% vs 34%). No severe adverse effects was reported by patients of any group.Thalidomide showed a persistent significant Hb-increment and transfusion independence in Hb E-β thalassemia patients compared to HU.

Immune suppression is a crucial pillar for treatment of aplastic anemia. Cyclosporine monotherapy is an easily available, affordable therapeutic option with good safety profile. This prospective study was conducted over a period of 2 years from June 2012 to July 2014. The diagnosis and response to treatment of aplastic anemia was established as per published criteria. Follow up was done at 3 and 6 months in order to assess the response. 57 patients of acquired aplastic anemia with median age of 37 years (6 to 81 years) were included in the study. 35 (62 %) cases were severe aplastic anemai, 16 (28 %) non severe aplastic anemia and 6 (10 %) were very severe aplastic anemia. At 3 months overall response rate (OR) was 7 (14 %) and at 6 months the OR rate of 11 (19.6 %) was achieved. Transiently raised creatinine, liver function abnormality and gum hypertrophy were the main side effects observed in this cohort. Oral cyclosporine monotherapy at dose of 5 mg/kg/day is a relatively safe treatment option for resource poor patients with aplastic anemia.

Background: Rarity of rare bleeding disorders (RBDs) is attributed to very low incidence based on ethnicity, distribution and complexity of diagnostic tools. There is scarcity of data on RBDs from Eastern India. This study aims to understand the distribution of RBDs in this region for better patient care.Methods: Retrospective study conducted on patients presented with symptoms/signs of bleeding from January 2018 to December 2019 (two years). Patients having bleeding manifestations from causes other than inherited coagulation disorders, IPDs, and vWD were included. Complete haemogram, peripheral blood smear, coagulation tests, vWD assay and platelet function tests were carried out.Results: Out of 2415 patients, 1570 were included after exclusion of other diseases. Immune Thrombocytopenia (ITP) and Hemophilia being excluded, 40/1570 (2.5%) patients were found to have RBDs. Median age was 11.6 years (range, 1.25-40 years). Most common was IPDs (2.9%, n=19); Glanzmann thrombasthenia was commonest (1.5%, n=10). vWD was second (1.7%, n=11) in occurrence; vWD type-III was the commonest subtype (0.9%, n=6). ICDs comprised 1.5% (n=10) of all patients; congenital afibrinogenemia (0.6%, n=4) was most common. Other ICDs prevalent in this cohort included FVII, FV, FX and FXIII deficiencies. Most common presentations were ecchymoses or prolonged bleeding from cut injuries or menorrhagia.Conclusions: RBDs may present in varied ways and are often difficult to diagnose correctly. Distribution of RBDs in this patient cohort differs from published literature from Western studies.

Background Burkholderia cepacia, an aerobic gram-negative bacillus, is a frequent colonizer of fluids used in the hospital ward. It poses little risk of infection to healthy people; however it is a known important opportunistic pathogen causing morbidity and mortality due to its intrinsic resistance to most of the antibiotics in hospitalized patients. Small hospital outbreaks are frequent. B. cepacia may occur as an opportunistic infection in hemato-oncology patients. Here we present an outbreak of Burkholderia cepacia infection in hematology ward of our institute. Methods Febrile episodes as defined by IDSA guideline, 2010 were followed, and blood for culture and sensitivity was sent in all the events. The culture was done by an automated method using Bactalert 3d Biomeriux & sensitivity pattern by Microscan Siemens method and subsequently detected by PCR based method. Results During September 2016 to February 2017 (six months), a total of 498 blood cultures were sent during febrile episodes. Out of which 60 (12%) came out to be positive for different microorganisms. Out of all positive cultures, Burkholderia cepacia was detected in 29 (48%) patients, which reduced drastically following the change in antibiotic administration practice. All isolates showed sensitivity to pipercillin+tazobactum, cefoperazone+sulbactum, fluoroquinolones, cotrimoxazole and carbapenems and resistance to polymyxin B and colistin. With timely intervention by appropriate intravenous antibiotics as per culture sensitivity result and change in antibiotic preparation practice, overall mortality was low 1 (4%) out of 29 culture positive episodes. Conclusion Change of antibiotic preparation practice was the key to control this outbreak, and overall mortality was low.

Thrombopoietin (THPO), also known as megakaryocyte growth and development factor (MGDF), is a cytokine involved in the production of platelets. THPO is a glycoprotein produced by liver and kidney. It regulates the production of platelets by stimulating the differentiation and maturation of megakaryocyte progenitors. It acts as a ligand for MPL receptor, a member of the hematopoietic cytokine receptor superfamily and is essential for megakaryocyte maturation. THPO binding induces homodimerization of the receptor which results in activation of JAKSTAT and MAPK signaling cascades that subsequently control cellular proliferation, differentiation and other signaling events. Despite the importance of THPO signaling in various diseases and biological processes, a detailed signaling network of THPO is not available in any publicly available database. Therefore, in this study, we present a resource of signaling events induced by THPO that was manually curated from published literature on THPO. Our manual curation of thrombopoietin pathway resulted in identification of 48 molecular associations, 66 catalytic reactions, 100 gene regulation events, 19 protein translocation events and 43 activation/inhibition reactions that occur upon activation of thrombopoietin receptor by THPO. THPO signaling pathway is made available on NetPath, a freely available human signaling pathway resource developed previously by our group. We believe this resource will provide a platform for scientific community to accelerate further research in this area on potential therapeutic interventions.

A white light emitting diode (LED) does not offer uniform irradiance in the visible region of electromagnetic spectrum. However, the additional irradiance in the blue regions is found to be complementary of the absorption spectrum peaks of human blood. Here, we have utilized such spectral characteristics of a typical white LED light as a source of white light interferometry for the measurement of thickness of blood film, additional irradiance of the LED spectrum is supposed to take care the imaginary component of refractive index of human blood. The thickness of blood duly measured by our developed technique is found to be essentially dictated by mean diameter of red blood cells as depicted by dynamic light scattering (DLS) studies on the corresponding blood samples. This technique can also follow the changes of the red blood cells diameter in isotonic, hypotonic and hypertonic solutions consistent with DLS studies on corresponding blood samples. Even quantitative agreement of the measured thickness of the blood film with the values obtained from DLS studies can be made on taking the surface roughness of the glass substrates (cover slips) as measured from Field Emission Scanning Electron Microscopy (FESEM). We have also proposed a low cost version of our developed technique, where, the relatively expensive spectrograph has been replaced by a band pass filter wheel.

Anemia severely and adversely affects human health and socioeconomic development. Measuring hemoglobin with the minimal involvement of human and financial resources has always been challenging. We describe a translational spectroscopic technique for noncontact hemoglobin measurement at low-resource point-of-care settings in human subjects, independent of their skin color, age, and sex, by measuring the optical spectrum of the blood flowing in the vascular bed of the bulbar conjunctiva. We developed software on the LabVIEW platform for automatic data acquisition and interpretation by nonexperts. The device is calibrated by comparing the differential absorbance of light of wavelength 576 and 600 nm with the clinical hemoglobin level of the subject. Our proposed method is consistent with the results obtained using the current gold standard, the automated hematology analyzer. The proposed noncontact optical device for hemoglobin estimation is highly efficient, inexpensive, feasible, and extremely useful in low-resource point-of-care settings. The device output correlates with the different degrees of anemia with absolute and trending accuracy similar to those of widely used invasive methods. Moreover, the device can instantaneously transmit the generated report to a medical expert through e-mail, text messaging, or mobile apps. (C) 2017 Society of Photo-Optical Instrumentation Engineers (SPIE)