Background/Objectives: Chronic kidney disease (CKD) is commonly complicated by anemia resulting from impaired erythropoietin (EPO) production, iron dysregulation, and chronic inflammation. Erythroferrone (ERFE) and hepcidin are key regulators of erythropoiesis and iron metabolism, but their interaction in CKD remains incompletely understood. This study aimed to examine the associations among ERFE, hepcidin, EPO, and hemoglobin, and to determine whether these markers independently relate to anemia severity in CKD. Methods: This cross-sectional case–control study included 126 patients with CKD (stages 2–5) and 33 age- and sex-matched healthy controls. Laboratory parameters, including hemoglobin, ferritin, transferrin saturation (TSAT), EPO, ERFE, hepcidin, and renal indices (eGFR, BUN, creatinine), were analyzed. Group differences were assessed using ANOVA or Kruskal–Wallis tests with post hoc analyses, and trends were evaluated using the Jonckheere–Terpstra test. Age- and sex-adjusted correlations and multivariable linear regression identified independent associations with hemoglobin. Results: Patients with CKD were older (61.2 ± 14.8 vs. 33.4 ± 10.7 years, p < 0.001) and had lower hemoglobin (11.8 ± 1.9 vs. 13.5 ± 1.4 g/dL, p < 0.001) and higher ferritin levels (245 (110–470) vs. 105 (40–240) ng/mL, p = 0.002) compared with controls. eGFR declined progressively across CKD stages (median (IQR): 73 (64–86) to 12 (7–17) mL/min/1.73 m2, p-trend < 0.001). ERFE and hepcidin showed increasing trends with advancing CKD (p-trend = 0.031 and 0.047, respectively). Hemoglobin correlated negatively with ERFE (r = −0.40, 95% CI: −0.53 to −0.26, p < 0.001) and positively with eGFR (r = 0.42, 95% CI: 0.28–0.54, p < 0.001). In adjusted regression analysis, ERFE (β = −0.29, 95% CI: −0.41 to −0.18, p < 0.001) and eGFR (β = 0.25, 95% CI: 0.13–0.37, p < 0.001) remained independently associated variables of hemoglobin (adjusted R2 = 0.47). Conclusions: Anemia severity in CKD is independently associated with both renal dysfunction and higher ERFE concentrations, suggesting a disrupted ERFE–hepcidin regulatory balance. These findings provide hypothesis-generating insights into the complex interplay between iron metabolism and erythropoiesis in CKD. Validation in larger, multi-center longitudinal studies that include inflammatory markers is warranted.
Central nervous system invasive aspergillosis is a rare fatal infection responsible for the majority of brain lesions in immunocompromised patients. A 56-year-old man with diabetes mellitus and non-Hodgkin's lymphoma was admitted to the emergency department with a diagnosis of pneumonia-related sepsis. At presentation, cranial computed tomography (CT) and magnetic resonance imaging were normal, but thoracic CT revealed right lung pneumonia, and antibiotic therapy was initiated. Control CT scans performed on the 13th day of admission because the patient had subsequently become hypotensive and somnolent revealed halo signs in the lungs and multiple hypodense lesions within the cerebrum, consistent with invasive aspergillosis. A post-contrast cranial CT scan also revealed vascular enhancement within the hypodense lesions, known as the central vascular sign. In conclusion, central nervous system aspergillosis can be diagnosed by means of tubular enhanced focuses in hypodense lesions on contrast-enhanced CT scans.
Background and Objectives: Acute myeloid leukemia and myelodysplastic syndrome are both clonal hematologic malignancies that primarily affect older adults. Current treatments for AML/MDS are both limited in number and efficacy. This study aims to evaluate venetoclax-based therapies in AML/MDS, focusing on overall survival and recurrence-free survival rates, and to expand real-world data on its use. Materials and Methods: Clinical and laboratory data on patients with AML/MDS aged 18≥ treated with venetoclax between January 2019 and July 2022 were included. Survival analysis was calculated based on the period from 2019 to December 2023. Results: A total of 161 AML and 40 patients with MDS were included. The median age was 63.53 ± 15.30 years for AML and 70.12 ± 10.21 years for MDS. In both groups, over 55% are male. A total of 77.6% of patients with AML and 75% of patients with MDS received treatment prior to venetoclax. Venetoclax was administered in combination with azacitidine to 84.5% of AML and 67.5% of MDS. The relapse rate in AML is approximately 15%. Overall, the 2-year survival rate is 46% and 18.73 months. The overall CR/CRi rate for patients with AML is 49.1%, while for patients with MDS, it is 50%. The 2-year survival rate for patients with MDS is 52.7%. The 2-year RFS rate was 75.5% for AML and 90.9% for MDS. The relapse rate in AML is approximately 15%. The percentage of adverse events leading to treatment discontinuation among those with grade 3–4 toxicity is low; 26.7% for AML (n = 43) and 15% for MDS (n = 6). Conclusions: Our real-world data demonstrate that venetoclax has the potential to improve overall survival rates when used in combination with HMAs and supports its use in patients with AML/MDS.
POEMS syndrome is characterized by small plasma cell clones and high serum VEGF levels. It causes M protein-related neuropathy and is considered a paraneoplastic syndrome.
Aim: Although loss of appetite in iron deficiency anemia (IDA) and weight gain during treatment are common complaints, there are very few studies in adults. This study aimed to determine the levels of ghrelin, one of the appetite-related hormones, and hepcidin, one of the main regulators of iron metabolism, in IDA, and to examine the effects of treatment on weight gain and the levels of these hormones. Material and Methods: Eighty-seven adult patients with IDA and a control group of 50 healthy volunteers were included in the study. Anthropometric measurements and blood samples were obtained from the patient and control groups before treatment, and repeated after treatment in the IDA group. Results: No significant difference was found in terms of weight, body mass index (BMI), and waist-to-hip ratio between groups but there was a significant increase in weight and BMI, in the patient group after treatment (both p
Objective: Multiple myeloma (MM) is a malignant hematological disease and anemia is observed in the majority of patients. Hepcidin, Growth differentiation factor-15 (GDF-15), soluble transferrin receptor (sTfR) have been investigated in many forms of anemia, especially in chronic diseases and cancers. However, there are few studies investigating their role in MM. We aimed to determine the relationship between hepcidin, sTfR and GDF-15 levels in MM patients and their clinical features such as anemia parameters, disease stage and overall survival. Method: Hepcidin, sTfR and GDF-15 levels, as well as clinical and anemia-related parameters, were analyzed in newly diagnosed MM patients and healthy volunteers. Result: Although MM patients had significantly lower Hb and Hct levels compared to the control group, none of the GDF-15, hepcidin and sTfR levels showed a significant difference between the groups. Among MM patients, we found that the anemic subgroup had significantly lower hepcidin levels than the non-anemic subgroup. GDF-15, hepcidin and sTfR levels showed weak or moderate positive correlation with each other, while GDF15 was positively correlated with creatinine and sTfR levels were correlated with many parameters such as LDH, CRP, ferritin, albumin, creatinine, Hb and ISS, all of which weak. None of the levels of GDF-15, hepcidin and sTfR had a significant effect on survival. Conclusion: We suggested that these mediators may play a role in anemia of MM but there is not a clear relashionship as in chronic disease anemia, there may be different mechanisms according to the characteristics of the patient groups.
The most common blood disease worldwide is anemia, defined by the World Health Organization as a condition in which the red blood cell count or oxygen-carrying capacity is insufficient. As both a disease and a symptom, this condition affects the quality of life. Early and correct diagnosis of the type of anemia is vital in terms of patient treatment. The increasing number of patients and hospital priorities, as well as difficulties in reaching medical specialists, may impede such a diagnosis. The present work proposes a system that will enable the recognition of anemia under general clinical practice conditions. For this system, a model constructed using four different artificial learning methods. Artificial Neural Networks, Support Vector Machines, Naïve Bayes, and Ensemble Decision Tree methods are used as classification algorithms. The models are evaluated with a dataset of 1663 samples and used 25 attributes, including hemogram data and general information such as age, sex, chronic diseases, and symptoms to diagnose 12 different anemia types. Data are collected by examining patient files at a university hospital in Turkey. In addition to all the data used by the doctors, the model also utilized eight different datasets created via particular feature selection techniques. The interface is designed to provide decision support to both medical consultants and medical students. Data are classified using the four different algorithms and an acceptable success ratio is obtained for each. Each model is validated using Classification Error, Area Under Curve, Precision, Recall, and F-score metrics in addition to Accuracy values. The highest accuracy (85.6%) achieved using Bagged Decision Trees, followed by Boosted Trees (83.0%) and Artificial Neural Networks (79.6%).
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare X-linked genetic disorder. On the contrary to its name, it is a multisystemic disease and various symptoms other than hemoglobinuria could be occurred. It could be life threatening especially because of thromboembolic events. In the last decade, a terminal complement inhibition with eculizumab approved with promising results for PNH patients. We conducted this study to evaluate the long term experience of eculizumab therapy from Turkey for the first time. Our cohort included 138 patients with PNH treated with eculizumab between January 2008 and December 2018 at 28 centers in Turkey. Laboratory and clinical findings at the time of diagnosis and after eculizumab therapy were recorded retrospectively. The median age was 39 (range 18-84) years and median granulocyte PNH clone size was 74% (range 3.06-99.84%) at the time of diagnosis. PNH with bone marrow failure syndrome was detected in 49 patients and the rest of 89 patients had classical PNH. Overall 45 patients (32.6%) had a history of any prior thrombotic event before eculizumab therapy and only 2 thrombotic events were reported during the study period. Most common symptoms are fatigue (75.3%), hemoglobinuria (18.1%), abdominal pain (15.2%) and dysphagia (7.9%). Although PNH is commonly related with coombs negativity, we detected coombs positivity in 2.17% of patients. Seven months after the therapy, increased hemoglobin level was seen and remarkably improvement of lactate dehydrogenase level during the treatment was occurred. In addition to previous studies, our real life data support that eculizumab is well tolerated with no serious adverse events and improves the PNH related findings.
Deciding on the diagnosis of the disease is an important step for treating the patients. Also, the numerical value of blood tests, the personal information of patients, and most importantly, an expert opinion is necessary to diagnose a disease. With the development of technology, patient-related data are obtained both rapidly and in large sizes. Deep learning methods, which can produce meaningful results by processing the data in raw form, are beginning to give results that are close to human opinion nowadays. The present work is aimed to develop a system that will enable the diagnosis of anemia in general practice conditions due to the increasing number of patients and the intention of the hospitals, as well as the difficulties in reaching the expert medical consultant. The main contribution of this work is to make a diagnosis like a doctor with the data as the way the doctor uses it. The data set was obtained from the actual hospital environment and no intervention, such as increasing or decreasing the number of data, increasing or decreasing the number of attributes, reduction, integration, imputation, transformation, or discretization, has been made on the incoming patient data. The original hospital data are classified for the diagnosis of anemia types and the accuracy of 84,97% achieved by using a deep learning algorithm.
Objective: Bone marrow aspiration and biopsy (BMAB) is an essential tool for diagnosis of hematological disorders. The most frequent complaint after BMAB is pain but the severity of this pain is described very different among patients. We investigated factors predicting this pain focusing on the role of state and trait anxiety. Methods: One hundred and ten adult patients undergoing BMAB, were informed adequately and assessed with "The State-Trait Anxiety Inventory" (STAI) before the procedure. In this Likert-type inventory, State Anxiety Scale evaluates the current state of anxiety, asking how respondents feel "at that moment". The Trait Anxiety Scale evaluates relatively stable aspects of "anxiety proneness," including general states of confidence, calmness, and security. After the biopsy, pain was measured with visual analog scale. Results: Most of the patients (71.8%) described mild pain but moderate to severe pain were significantly more frequent in both high state and trait anxiety groups. Pain severity had a positive but weak correlation with trait anxiety but not with state anxiety. The described pain level was associated with older age but was not with indication of biopsy, performance status, comorbidities or previous BMAB experiences. Conclusions: Results of our study made us thought that a good communication with the patient and talking about possible outcomes days before procedure might play a role reducing his or her anxiety but because age and trait anxiety cannot be changed by using fast acting anxiolytic drugs, advantage of premedication with anxiolytics in order of reducing pain, would be limited.
Catastrophic antiphospholipid syndrome is a rare but rapidly progressing form of antiphospholipid antibody syndrome with high mortality. The syndrome causes multiorgan failure associated with diffuse microthromboses. Necrotic-appearing ecchymotic lesions emerging from the distal aspect of both lower extremities and progressing to the upper leg region, and to the upper extremities developed in a 58-year-old woman. Histopathological examination of the biopsy specimen revealed intravascular microthrombi in the dermis. Laboratory findings for anti-cardiolipin antibodies IgM and lupus anticoagulant resulted positive. Lung and kidney involvement was observed. The clinical course progressed very quickly, and catastrophic antiphospholipid syndrome was diagnosed with these findings. Two-thirds of catastrophic antiphospholipid syndrome cases develop due to secondary causes, the most common being infections. Catastrophic antiphospholipid syndrome may also accompany autoimmune diseases, particularly systemic lupus erythematosus. This report is presented to emphasize that Sjogren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.
Infectious mononucleosis (EM) is a disease characterized by irradiation of the Epstein-Barr virus (EBV). The clinical manifestation is characterized by tonsillopharyngitis, lym-phadenopathy and atypical lymphocytosis. We describe a young patient developing hemolytic anemia during follow-up in our clinic due to tonsillopharyngitis secondary to EBV and general condition disorder.
ÖZETGİRİŞ ve AMAÇ: Akut myeloid lösemi(AML) yaşlı populasyonda sıklığı artan hematolojik malignitedir.Düşkün ve yaşlı hastalarda düşük yoğunluktaki tedaviler ve destek tedavisi uygun seçenekler olarak öngörülmektedir.On yıllarda hipometile edici ajanların kullanımı ile tedavi alanında olumlu gelişmeler yaşanmıştır.Çalışmamızın amacı merkezimizde ileri yaş AML hastalarımızda tedavi protokolu olarak uyguladığımız etoposit ve sitozin arabinozid(AraC) ile güçlendirilmiş azasitidin(AZA
Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease which is characterized with complement mediated intravascular hemolysis, bone marrow failure and thrombosis. The prevelance of PNH is estimated 1 to 16 cases per million in USA. X-linked somatic mutation of phosphatidylinositol glycan‐A (PIGA) gene in hematopoetic stem cells causes an impairment of glycosylphosphatidylinositol (GPI) anchored proteins. The absence of GPI‐dependent molecules that are CD55 (decay accelerating factor; DAF) and CD59 (membrane inhibitor of reactive lysis; MIRL), normally protect the cell from complement‐mediated hemolysis by preventing the formation of the membrane attack complex. The diagnosis is based on flow cytometry which can detect the deficiency of these two complement regulary proteins. We report a retrospective analysis of demographic and clinical characteristics of PNH patients from different centers. Material and methods: We conducted a retrospective analysis of the patients' recorded data. Patients' demographics, medical and treatment history, comorbid conditions, PNH clone size, disease characteristics and outcomes, symptoms, PNH-specific treatments, PNH-related events, morbidity (including myeloproliferative disease, other malignancies, and infections), mortality. Clinical data captured include lactate dehydrogenase (LDH) levels, PNH clone size, hemoglobin levels, thrombotic events, renal functional tests at the time of diagnosis, and other laboratory data. Specific information collected for eculizumab-treated patients includes dosage and dose adjustments and blood cell counts, reticulocyte count and LDH level after eculizumab treatment. Results: 138 patients were included from 28 different centers. All patients were diagnosed by flow cytometry for GPI-linked antigens on red cells and neutrophils. The number of male (69/138) and female (69/138) patient was equal and the median age was 41 years. Median hemoglobin (hb) level was 8.75±2.13 gr/dL; Platelet (plt) level was 131× 109/L at the time of diagnosis. Overall, 49(35,5%) of the patients had been diagnosed with bone marrow failure, including aplastic anemia or hypoplastic anemia (n=31; 22,5%), myelodysplastic syndromes (n=18; 13%). A history of any prior thrombotic event was reported in 45 patients (32,6%). At the time of analysis, 12 (8,7%) patients had pulmonary hypertension. The median granulocyte and monocyte clone size was 63,6% (±32.26) and 66.76 ±28.75 respectively. Fatigue (58%) is the most commonly reported symptom and abdominal pain was seen in 8% of patients. After the eculizumab therapy, the median time for normalization of Hb and LDH level were 7 and 14,6 months, respectively. There was no correlation between thrombosis and clone size, hb, plt, LDH level at the time of diagnosis. LDH level was higher in fatigue patients compared with the patients who were not fatigue (p=0.021). Discussion: PNH is a clonal but non malignant disease that is very rare and knowledge on large case series is really limited. The clinical findings and symptoms could be variable and unfortunately it takes very long time to diagnose because of unawareness of physicians. Eculizumab is a good option in treatment of PNH for improving symptoms of intravascular hemolysis but we still need better understanding of thrombosis mechanism of PNH to better management. In the future, novel inhibitors of the alternative pathway of complement will be used to improve survival and quality of life for PNH patients. Disclosures No relevant conflicts of interest to declare.
Aim: Rapid prediction of prognosis is helpful in reflecting the disease severity and patient mortality. This is especially important in critically ill elderly patients who have high mortality risk. This study aimed to investigate the effects of admission laboratory results and medical histories on the prediction of prognosis in critically ill elderly patients. Material and Methods: Patients who were >= 65 years and admitted to a medical intensive care unit (ICU) between 2011 and 2013 were retrospectively analyzed. Results: The study included 449 patients and mortality rate was 47.4%. Nonsurvivors had lower pH, HCO3 and albumin levels, and lower hematocrit and platelet counts, but higher aspartate aminotransferase, alanine aminotransferase, C-reactive protein (CRP), creatinine, phosphorus, magnesium and bilirubin levels, and higher leukocyte count than survivors. The rates of chronic kidney disease, being in a bedridden state and having cardiopulmonary resuscitation (CPR) before ICU admission were significantly high in nonsurvivors. Multivariate analysis showed that pH <7.20, albumin <= 2 gr/dL, low hematocrit and high CRP levels, high leukocyte count, bedridden state, and CPR were mortality predictors. After including the admission diagnoses and endotracheal intubation into the model, pH <7.20 (odds ratio [OR], 4.31; 95% confidence interval [CI], 1.59-11.70), albumin <= 2 gr/dL (OR, 3.61; 95% CI, 0.99-13.03), hematocrit level (OR, 0.94; 95% CI, 0.91-0.99) and leukocyte count (OR 1.06; 95% CI, 1.01-1.11) retained their prognostic importance for mortality. Conclusions: Severe acidemia, low albumin and hematocrit levels, and high leukocyte count at admission help clinicians to foresee the prognosis in severely ill elderly patients. They keep their importance even in the presence of other fundamental mortality predictors.