Indian Journal of Hematology and Blood Transfusion最新文献

A 23-year-old man presented with worsening fatigue, coarse facial features, digital clubbing, and splenomegaly. Laboratory tests revealed severe anemia and bone marrow fibrosis. Genetic analysis identified a pathogenic mutation in the SLCO2A1 gene, confirming a diagnosis of autosomal recessive primary hypertrophic osteoarthropathy type 2 (PHOAR2). Treatment with Etoricoxib, a COX-2 inhibitor, led to gradual improvements in fatigue, reduction of spleen size, and increased hemoglobin levels over six months. This case highlights the association between elevated prostaglandin E2 levels and myelofibrosis in PHOAR2, emphasizing the potential of COX-2 inhibitors in managing symptoms.

Sickle cell disease is an inborn genetic blood disorder that is usually found among residents of Africa, Middle-East Gulf countries, and India. There has been increased knowledge and awareness of the range of neurological complications of Sickle cell disease (SCD) over the past 20 years. Silent neurologic involvement may occur in SCD and there's an opportunity of subclinical involvement in these patients presenting with vasoocclusive crisis. The case-control study was carried out after getting the institutional ethical clearance and obtaining consent from the patients and the controls. They were subjected to nerve conduction studies. Nerve conduction study was performed on median and tibial nerves for motor nerves, and median and sural for sensory nerves unilaterally. The nerves of the upper limb showed changes in neuropathy. The lower limb nerves were completely normal. In our study, we found out that Distal motor latency (DML) is prolonged in the Median motor nerve in cases as compared to controls suggesting motor neuropathy, and Sensory nerve action potential (SNAP) is reduced in the median sensory nerve suggesting sensory neuropathy. The nerve conduction studies in the tibial nerve were normal, suggesting subclinical neuropathy could be present in the patients with sickle cell presenting as mononeuropathy or mononeuritis multiplex. Our study also found a statistically significant correlation of neuropathy with the increasing number of hospital admissions for the vaso-occlusive crisis. Subclinical neuropathy is another manifestation of nervous system in patients with SCD and one should give adjuvant therapy of membrane stabilizing agents for the relief of pain. Detailed studies of this complication must be required further to evaluate its significance in clinical practice. We also conclude the aches and the pain the patient of sickle cell disease complains could not be always due to vaso-occlusive crisis, it could be the neuropathic pain which might mimic the ischemic pain.

Leukemia is one of the most common hematological malignancies. Cutaneous manifestations of leukemias consist of two groups: specific and non-specific. While the drug reactions, opportunistic infections due to myelosuppression are non-specific skin findings of leukemias, leukemia cutis is a specific sign of cutaneous involvement. In this retrospective study, we included patients over the age of 18 who were diagnosed with acute myeloid leukemia (AML) and underwent histopathological examination due to dermatological complaints. A total of 21 patients were included. Histopathological examination results were consistent with myeloid sarcoma, erythema nodosum, cutaneous drug eruption, necrosis due to vascular damage, cutaneous vasculitis, graft versus host disease, Sweet syndrome and viral infection. Dermatological examination plays an important role in AML patients. Skin biopsy and immunohistochemical examination should be performed to make early diagnosis of skin metastasis of leukemias and paraneoplastic syndromes to reduce the mortality and morbidity in AML patients.

Transfusion of Packed RBC units (PRBC) in multi-transfused patients often results in a progressive rise in transfusion requirements. To evaluate the procoagulant potential of PRBC in multi-transfused patients. We conducted this prospective cohort study (2020-2022) on multi-transfused patients (n = 31) having a lifetime transfusion of ≧ 4 units PRBCs. Biochemical parameters pre-, post-transfusion (≤ 24 hours) samples included, highly sensitive C reactive protein (HsCRP) for inflammation, LDH for hemolysis, tissue plasminogen activator (tPA), Antithrombin 3 (ATIII) levels as coagulopathy, anti-hemostatic marker and blood microparticle (MPs) (flowcytometry) as Annexin 5+ (AV+) events for TF. We performed Wilcoxon signed rank test (P≤ 0.05), pre-, posttransfusion (P≤ 0.05) and association pre- and posttransfusion by Spearman's rho correlation coefficient (CC), Linear regression (r²). Median (95% CI; P≤ 0.05) biochemical parameters pre-, posttransfusion, tPA {7.88 (2.63-20.44); 14.40 (5.29-27.08)} (P = 0.012) and ATIII {1299.66 (1078.5-1362.5); 1358.92 (1216 -1384.3)} (P = 0.011) respectively. A pre-, posttransfusion comparison of HsCRP (P = 0.45); LDH (P = 0.87) and MPs (number of events) (P = 0.54) pre-, posttransfusion were not significantly different. We observed a significant CC pre-posttransfusion HsCRP (0.61, P < 0.01); ATIII (0.480, P = 0.006); tPA (0.807, P < 0.01); MPs (0.625, P < 0.004) and r² tPA (posttransfusion) (dependent variable) tPA (pre-transfusion), ATIII (pre-transfusion) (predictor variables) r² = 0.85 (P < 0.01); ATIII (posttransfusion) (dependent variable) and ATIII (pre-transfusion); LDH (pre-transfusion) (predictor variable) r² = 0.45 (P = 0.026). A significant differences tPA, ATIII (pre-posttransfusion) and an association 'pre-posttransfusion' may be attributed to PRBC transfusions. tPA levels with corresponding changes in ATIII indicate coagulopathic response, following PRBC transfusions.

Supplementary information: The online version contains supplementary material available at 10.1007/s12288-025-01978-0.

Nutritional anaemias are on the rise in India despite several control programs. Haemoglobin synthesis needs iron, vitamin B12, folic acid, and a source of complete protein (containing all essential amino acids). While we were focusing on iron deficiency only, in reality, one or more nutrients needed for haemoglobin synthesis may be missing from the diet due to lack of awareness, empowerment, and marginalisation issues. Therefore, nutritional anaemias represent the tip of the iceberg of clinical and subclinical malnutrition in our scenario, in a vast majority of patients. There are several myths on nutrition and a balanced diet, rooted in cultural, religious, and regional factors. In the developed countries, these nutritional deficiencies are more often due to physiologically or pathologically increased need or due to malabsorption or increased losses of the nutrients involved or blood loss, which are equally important concerns in our scenario too. To make a beginning, we need to work on creating awareness on a balanced diet, removing false beliefs and wrong practices related to food items. Equally important for a sustainable solution is to empower the people through social, economic, and agricultural reforms, and policy changes, for consuming a balanced diet.Other social aspects of malnutrition, including poor management of human and financial resources, wrong influences on the people by advertisements, and the growing fast-food culture, need to be addressed. Patients of anemia need to be evaluated with proper investigations in an algorithmic approach, especially when nutritional supplementation is producing sub optimal results. Other causes of anemia including hemoglobinopathies, malnutrition associated, anemia of chronic disease need to be investigated. For iron deficiency, treating the underlying cause of excessive blood loss is essential. All this will go a long way to achieve the goal of anaemia-free India.

To analyse the trends in drug susceptibility patterns (DSP) of gram- negative bacilli (GNB) in setting of febrile neutropenia in hematology-oncology patients. This retrospective study (done from April 2019-April 2024) compares clinical (Charlson co-morbidity index [CCI], Sequential organ functional assessment [SOFA] score, Pitts bacteraemia score, and all cause 30 day mortality) parameters and trends in DSP of GNBs isolated from blood cultures of hematology-oncology patients. Various AMS activities, including restriction on use of high end antibiotics, cessation of antibiotics in patients with pre-neutropenic fever, involvement of an infectious diseases physician for management of multi-drug resistant (MDR) infections and improvement in diagnostics, among others, were done during this time period. In 535 patients, 601 different GNB bacteraemia episodes were identified. Time series analysis showed that carbapenem resistance in Klebsiella pneumoniae and Escherichia coli decreased from 90.3 to 48.1% (r = -0.961, p = 0.0006) & 54.5-27.7% (r = -0.882, p < 0.001) respectively, DTR (difficult to treat) rates in Pseudomonas aeroginosa fell from 83.3 to 10% (r = -0.716, p- 0.036). This study provides evidence that resistance in GNB can be curtailed by AMS activities even in highly vulnerable population like hematology-oncology.

Purpose: Assessment of the effect of cytotoxic chemotherapy on the indices of iron homeostasis to elucidate the aetiology of anaemia in breast cancer.

Methods: Forty-two newly diagnosed breast cancer patients with Hb > 10 g/dL were recruited. Iron indices were estimated before and after neoadjuvant chemotherapy (NACT). Anaemia of chronic disease (ACD) and anaemia of chronic disease + iron deficiency anaemia (IDA) were identified by sTfR, sTfR index, and algorithm by Skikne et al.

Results: Anaemia was 52% at the presentation time, which increased to 93% at the end of NACT. Among the anaemic breast cancer patients, 75% belonged to the group of ACD + IDA, and only 25% belonged to the group of ACD at the time of presentation. After NACT, the number of patients in the ACD + IDA group significantly increased to 93%, while the members of the ACD group significantly reduced to 7%. Ferritin, Hepcidin, Transferrin, sTfR, and sTfR index increased after NACT.

Conclusion: The primary aetiology of anaemia among Indian breast cancer patients is a combination of ACD and IDA. Indian breast cancer patients were more vulnerable to developing iron deficiency anaemia when compared to anaemia of chronic disease during NACT.

Anemia is a common problem encountered by patients hospitalized in the intensive care unit (ICU). However, the relationship between transfusion and mortality has not been clearly determined. We aimed to investigate the effects of a restrictive transfusion practice on patient outcomes in the ICU. We enrolled 143 patients who were hospitalized in the ICU between August 2018 and August 2019. Patients were categorized by whether they had received transfusion during their stay. Transfusions were performed according to a restrictive transfusion policy with a hemoglobin (Hb) threshold of < 7 g/dL. Transfusion was required for 43% of the patients with a median of 3 units used. Transfused patients were older (79.3 vs. 74.6 years; p = 0.034), had greater length of stay (LOS) in the ICU (LOS-ICU: 51 vs. 9.5 days; p = < 0.001), were under invasive mechanic ventilation (LOS-IMV: 20 vs. 7.5 days; p = 0.026), and higher mortality rates (68.9% vs. 37.8%; p = < 0.001) than those who did not. Age (odds ratio [OR]: 1.03), sepsis or septic shock (OR: 2.74), placement of central venous catheter (OR: 6.36), and LOS-ICU (OR: 1.01) were defined as predictors of transfusion. However, after hierarchical logistic regression analyses, mortality rates were not associated with transfusion. The need for vasopressor use (OR: 17.3) and IMV (OR: 38.3) were predictors of mortality. Although transfused patients were more severely ill, whether they underwent transfusion or not did not correlate with increased mortality rates. Selecting a restrictive transfusion policy and shortening LOS-ICU might provide better patient outcomes. While this study supports restrictive transfusion policies, it primarily contributes region-specific data rather than novel or generalizable findings.

Estimation of haemoglobin (Hb) plays an important role in the medical examination of prospective blood donors. Various tests to estimate Hb are available in the market to screen for donor Hb with their own set of advantages and disadvantages. Each Blood centre chooses their test based on the availability of funds / resources, manpower and number of donations. Like any other test, Hb estimation tests also have various factors resulting in erroneous results. Identifying and preventing such factors is crucial in maintaining the safety of donor and the patient. This short research communication dwells upon such common factors influencing Hb estimation in the routine blood donor screening and what can be done to prevent such errors.