Indian Journal of Hematology and Blood Transfusion最新文献

There is paucity of data from low-middle income countries (LMIC) on osteonecrosis (ON) in survivors of childhood acute lymphoblastic leukaemia (cALL-survivors). We conducted this study to estimate prevalence of ON in cALL-survivors of Indian ethnicity and factors affecting it. This cross-sectional study enrolled cALL-survivors post completion of treatment. ON was estimated using magnetic resonance imaging of hip joint. Demographic, anthropometric, therapy/disease-related and biochemical/endocrine factors affecting calcium homeostasis were studied in two groups: with and without ON. Total of 61 out of enrolled 87 cALL-survivors with median age 118 months (range:84–283) were analysed after median 12 months (range:1–113) post completion of therapy. Two-third of the cohort was male and 41% were pubertal. 5/61 (8.2%) were found to have asymptomatic and non-traumatic ON. Three ON were grade II and two were grade III as per Niinimaki radiological classification. Cumulative doses (CD) of dexamethasone, glucocorticoids (GCs), L-asparginase, anthracycline and low serum vit D levels were associated with ON. Other demographic factors including age at diagnosis > 10 year, disease-related, therapy-related factors including cranial irradiation and biochemical/endocrine factors were not associated with ON. The median CD of dexamethasone (p = 0.004) and GCs (p = 0.008) were significantly high in group with ON. Median CD of methotrexate(p = 0.051), anthracycline(p = 0.058) and serum vit D levels(p = 0.054) along with serum alkaline phosphatase levels(p = 0.06) had a trend towards significance but were not statistically significant in ON group. Prevalence of ON of hip in our cohort of cALL-survivors was 8.2%. Higher CD of GCs appeared to be the most significant risk factor associated with ON in our cohort.

Introduction

Iron deficiency anemia (IDA) is a common health problem. The hepcidin hormone is the main regulator of systemic iron balance. The body responds to IDA by decreasing hepcidin. This study investigated how different iron supplementation regimens affect hepcidin levels in women with IDA. 87 female participants aged 18–45 years with hemoglobin < 10 g/dL and serum ferritin < 20 ng/ml were assigned to receive iron therapy every other day, once daily, or twice daily. Hemogram, serum iron, serum iron binding capacity, ferritin, hepcidin, and C-reactive protein values were measured at baseline and on the 15th and 90th days of treatment in all groups. On the seventh day, no significant difference was found between the once-daily and twice-daily groups (p = 0.42) in reticulocyte counts. By the 15th day, hemoglobin and MCV levels showed significant improvement in the twice-daily group compared to the other groups (p < 0.01). At the third month, ferritin levels were significantly higher in the twice-daily group compared to the every-other-day and once-daily groups (p = 0.03). No significant differences were observed in hepcidin levels at three months across all groups. The study concludes that twice-daily iron supplementation results in the most significant hematological improvements but with increased gastrointestinal side effects. These findings underscore the importance of tailoring iron dosing schedules to individual patient needs. In cases where rapid haemoglobin response is required, twice-daily dosing may provide superior results. Conversely, once-daily dosing may be preferred if tolerable anemia can be maintained. Every other day dosing, although associated with fewer side effects and better tolerability, may not provide adequate support for erythropoiesis.

Physical activity plays an important role in Hemophilic patients it provides multiple benefits to patients but in Hemophilia, patients instead of doing physical activity fear to do movement or any kind of exercise. Some associated factors that can prevent the patient from doing movement are pain, fear, or Kinesio-phobia and arthropathy. So, the study aimed to find the association between physical activity and fear of movement in Hemophilic patients. This cross-sectional study was done on 30 Hemophilics. All Hemophilic patients were males and between the age group of 18–60 years (mean = 29.36 ± 10.84). Data about physical activity and Kinesio-phobia was taken. The Tampa scale of Kinesio-phobia was used to determine the fear of movement. Physical activity was also determined. The results were analyzed by using the Mann–Whitney U test. There was no significant statistical correlation between physical activity and fear of movement in patients with mild and moderate hemophilia but there was a significant in severe hemophilic patients. High Kinesio-phobia levels (TSK score of ≥ 37) were present in 90% of patients. There is a higher rate of Kinesio-phobia in adult patients of Hemophilia so, education should be provided to patients about safe and fearless physical activity.

Capillary zone electrophoresis (CZE) is an easy to perform technique encompassing a wide range of clinical applications ranging from inflammatory, renal, hepatic, neurological disorders as well as hematological malignancies like Plasma cell neoplasms. The objectives of the study are to explore various patterns of serum protein abnormalities in different medical diseases ranging from systemic disorders to hematological malignancies coming to our institute and to correlate these electrophoretic abnormalities with clinic-hematological and biochemical parameters. The present study was conducted in the Department of Hematology of a tertiary care institute over a period of 20 months. Altogether 980 samples were received for serum protein electrophoresis (SPE) in clinically indicated cases. Detailed clinical history, hematological and biochemical reports were compiled and analyzed with SPE reports. SPE was performed by Minicap Flex piercing fully automated capillary electrophoresis system from Sebia France and results were interpreted. In the present study, out of 980 cases 630 (64.3%) were males and 350 (35.7%) were females with a M: F ratio of 1.9:1. Most of the cases were in the age group of 51–60 years (n-285, 47.4%) followed by 41–50 years (n = 188, 27%). The most common indication for SPE was Chronic kidney disease (CKD) that accounted for 50% of all cases, followed by Plasma cell neoplasms (40%). Acute kidney injury (AKI), nephrotic syndrome (NS), peripheral neuropathy, and unexplained anemia were the other indications. Most common SPE pattern obtained on CZE was that of polyclonal hypergammaglobulinemia accounting to 316 of all cases (32.2%), closely followed by 292 cases of chronic inflammatory pattern (29.7%). Distortion in gamma region was seen in 14.7% cases (145/980). A distinct M spike was seen in 57 cases (5.8%) suggesting a diagnosis of monoclonal gammopathy. A comprehensive look at all the protein fractions, their distortions and peaks along with clinico-biochemical and hematological correlation can help in reaching out to a correct diagnosis.

Antiphospholipid syndrome (APS) is an autoimmune disease with specific clinical features and the presence of antiphospholipid antibodies (aPL) like anti-beta2 glycoprotein 1 (anti-β2gp1), anti-cardiolipin antibody (aCL), and/or lupus anticoagulant (LA). The purpose of this study was to evaluate the laboratory profile of LA-positive cases and study its association with various clinical presentations. In this ambispective analytical study over 20 months, LA-positive cases (n = 167) from among 970 cases screened were included. Tests for LA were integrated dilute Russell’s Viper venom time (dRVVT) and silica clotting time (SCT) using screen-confirm procedure with mixing whenever necessary. The clinical profile and other investigations like aCL and anti-β2gp1were noted from records. The sensitivity of dRVVT and SCT for LA positivity were 78.4% and 79.4%, respectively. Based on the clinical presentation the cases were grouped as abortion, thrombosis, systemic lupus erythematosus (SLE) and others. The thrombotic group showed significantly higher (94%) dRVVT positivity, with an odds ratio of 5.56 (95% CI: 1.61 to 19.15). SCT, aCL, or anti-β2gp1 positivity did not show any significant risk association with thrombosis or abortion. SLE- group showed more frequent dual-LA (dRVVT and SCT) positivity. All groups showed higher anti-β2gp1 positivity than aCL. Persistent LA positivity after 12-weeks was more frequent with dual-LA positive (91.67%), double (LA + aCL/anti-β2gp1) and triple (LA + aCL + anti-β2gp1) positive cases (100%). Both dRVVT and SCT had comparable sensitivity. dRVVT positivity was significantly associated with increased thrombotic risk. Persistent LA positivity was more often seen with initially dual, double or triple LA positive cases.

We investigated whether reduced intensity-chemotherapy (IC) is associated with decreased toxicity and longer overall survival (OS) in elderly AML patients. Age-dependent dose-reduced IC was administered to 110 AML patients between 2004 and 2021. We assessed myelosuppressive toxicity, clinical efficacy, and safety of our regimen using the depth index (D-index). Patients of 66–79 years of age (younger elderly [YE], n = 52) and ≥ 80 years (older elderly [OE], n = 19) were compared to a control group of patients of ≤ 65 years of age (n = 39). Although no significant differences were observed in the number of days with neutrophil count < 500/µl, the D-index, or the onset of sepsis among the groups, OS significantly differed (median OS: control, 578 days [317 days-NA]; YE, 281 days [158–515 days]; OE, 185 days [72–373 days]; p = 0.0001). IC for elderly AML patients achieved negative treatment outcomes despite a reduction in myelosuppressive toxicity, with no data beyond a median OS of 14.7 months for Azacytidine + Venetoclax therapy in a phase 3 VIALE-A trial. Although the findings were negative, the present results provide insights into appropriate IC regimens for elderly AML patients in the future.

Purpose: This case series describes an increase in the incidence of cold agglutinin syndrome (CAS) in children in Singapore following relaxation of COVID-19-related non-pharmacological interventions (NPIs). Methods: Clinical data of patients diagnosed with CAS from November 2022 to October 2023 following the relaxation of NPIs in October 2022 were collected retrospectively. Data on the number of CAS cases diagnosed before the pandemic (2018–2019) and during the pandemic (January 2020-October 2022) were collected. Results: Thirteen patients were diagnosed with CAS from November 2022 to October 2023, as compared to zero cases diagnosed during the COVID-19 pandemic. There were also more cases than before the pandemic (one case in 2018 and three cases in 2019). The median age of the 13 patients was 4.6 years (range 1.8–12 years). The most commonly detected virus was rhinovirus/enterovirus (54%). Nine children required red cell transfusions. Five patients received corticosteroids. Conclusion: An increase in the number of pediatric CAS cases has been observed since the COVID-19 pandemic. This may be due to an overall increase in viral infections due to the immunity debt from COVID-19 related NPIs. Another possible explanation is the “hygienist theory, ” which postulates a causal relationship between the decline in infections and increase in immunological disorders.