Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike patients with beta (β)-thalassemia major, do not require regular transfusion therapy for survival. The most commonly investigated forms are β-thalassemia intermedia, hemoglobin E/β-thalassemia, and α-thalassemia intermedia (hemoglobin H disease). However, transfusion-independence in such patients is not without side effects. Ineffective erythropoiesis and peripheral hemolysis, the hallmarks of disease process, lead to a variety of subsequent pathophysiologies including iron overload and hypercoagulability that ultimately lead to a number of serious clinical morbidities. Thus, prompt and accurate diagnosis of non-transfusion-dependent thalassemia is essential to ensure early intervention. Although several management options are currently available, the need to develop more novel therapeutics is justified by recent advances in our understanding of the mechanisms of disease. Such efforts require wide international collaboration, especially since non-transfusion-dependent thalassemias are no longer bound to low- and middle-income countries but have spread to large multiethnic cities in Europe and the Americas due to continued migration.

/pdf/non-transfusion-dependent-thalassemias-46pe7uf4mg.pdf

Non-transfusion-dependent thalassemias

How I manage medical complications of β-thalassemia in adults.

Despite receiving no or only occasional blood transfusions, patients with non-transfusion-dependent thalassemia (NTDT) have increased intestinal iron absorption and can accumulate iron to levels comparable with transfusion-dependent patients. This iron accumulation occurs more slowly in NTDT patients compared to transfusion-dependent thalassemia patients, and complications do not arise until later in life. It remains crucial for these patients' health to monitor and appropriately treat their iron burden. Based on recent data, including a randomized clinical trial on iron chelation in NTDT, a simple iron chelation treatment algorithm is presented to assist physicians with monitoring iron burden and initiating chelation therapy in this group of patients. Am. J. Hematol. 88:409–415, 2013. © 2013 Wiley Periodicals, Inc.

/pdf/treating-iron-overload-in-patients-with-non-transfusion-3l9wy2ueg2.pdf

Treating iron overload in patients with non-transfusion-dependent thalassemia

Outcome prediction for patients with sepsis may be conductive to early aggressive interventions. Numerous biomarkers and multiple scoring systems have been utilized in predicting outcomes, however, these tools were either expensive or inconvenient. We performed a meta-analysis to evaluate the prognostic role of red blood cell distribution width (RDW) in patients with sepsis. The online databases of Embase, Web of science, Pubmed, Corchrane library, Chinese Wanfang database, CNKI database were systematically searched from the inception dates to June, 24th, 2020, using the keywords red cell distribution width and sepsis. The odds ratio (OR) or Hazards ratio (HR) with corresponding 95% confidence intervals (95%CI) were pooled to evaluate the association between baseline RDW and sepsis. A random-effects model was used to pool the data, and statistical heterogeneity between studies was evaluated using the I2 statistic. Sensitivity and subgroup analyses were performed to detect the publication bias and origin of heterogeneity. Eleven studies with 17,961 patients with sepsis were included in the meta-analysis. The pooled analyses indicated that increased baseline RDW was associated with mortality (HR = 1.14, 95%CI 1.09–1.20, Z = 5.78, P < 0.001) with significant heterogeneity (I2 = 80%, Pheterogeneity < 0.001). Similar results were found in the subgroup analysis stratified by site of infection, comorbidity, Newcastle-Ottawa Scale (NOS) score, study design, patients’ country. The predefined subgroup analysis showed that NOS score may be the origin of heterogeneity. For patients with sepsis, baseline RDW may be a useful predictor of mortality, patients with increased RDW are more likely to have higher mortality.

/pdf/prognostic-role-of-red-blood-cell-distribution-width-in-1l476yxu3k.pdf

Prognostic role of red blood cell distribution width in patients with sepsis: a systematic review and meta-analysis

Red blood cell distribution width (RDW), an integral parameter of the complete blood count (CBC), has been traditionally used for the classification of several types of anemia. However, over the last decade RDW has been associated with outcome in patients with several cardiovascular diseases including heart failure. The role of RDW in acute, chronic and advanced heart failure is the focus of the present work. Several pathophysiological mechanisms of RDW’s increase in heart failure have been proposed (i.e., inflammation, oxidative stress, adrenergic stimulation, undernutrition, ineffective erythropoiesis, reduced iron mobilization, etc.); however, the exact mechanism remains unknown. Although high RDW values at admission and discharge have been associated with adverse prognosis in hospitalized heart failure patients, the prognostic role of in-hospital RDW changes (ΔRDW) remains debatable. RDW has been incorporated in recent heart failure prognostic models. Utilizing RDW as a treatment target in heart failure may be a promising area of research.

/pdf/red-blood-cell-distribution-width-in-heart-failure-1hgcfb06.pdf

Red Blood Cell Distribution Width in Heart Failure: Pathophysiology, Prognostic Role, Controversies and Dilemmas

Despite evidence of considerable iron overload in transfusion-independent patients with β-thalassemia intermedia, data on hepatic outcomes remain scarce. We analyzed data from a cohort of 42 β-thalassemia intermedia adults followed for four years (median age 38years), and evaluated the association between longitudinal changes in serum ferritin levels and transient elastography values, a measure of hepatic stiffness predictive of fibrosis. We observed a significant increase in both serum ferritin levels (+81.2 [μg/l]/year) and transient elastography values in non-chelated patients (n=28) (+0.3kPa/year), with two patients worsening their fibrosis stage. Chelated patients (n=14) had a significant decrease in both measures (-42.0 [μg/l]/year and -0.9kPa/year, respectively), with two patients improving their fibrosis stage. There was a strong correlation between the rate of change in serum ferritin level and the rate of change in transient elastography value (R(2): 0.836, p<0.001) noted in both non-chelated and chelated patients. An association between iron overload status and hepatic disease merits further evaluation in this subset of transfusion-independent patients.

Longitudinal changes in serum ferritin levels correlate with measures of hepatic stiffness in transfusion-independent patients with β-thalassemia intermedia

Lymphocyte-to-white blood cells ratio in older patients experiencing a first acute heart failure hospitalization.

Hyponatremia: a challenge for internists.

Background: Red blood cell distribution width (RDW) is a risk factor related to adverse outcome in patients with heart failure (HF). Less is known about its influence in patients in their first hospitalization by HF.

 

Aims: Our objective was to investigate the prognostic role of RDW in elderly patients firstly hospitalized for acute HF.

Methods: We reviewed all patients ≥ 65 years old admitted to a tertiary care university hospital with a main diagnosis of acute HF during a two year period (January 2013 to December 2014). Patients were divided in two different groups according to admission RDW values (< or ≥ 15%).

Results: A total of 897 patients were included in the study. Mean age was 80.25 ± 7.6 years. Admission RDW was ≥ 15% in 474 (52.8%) patients, with a mean RDW of 15.5 % ± 2.3. Multivariate analysis confirmed the relationship between a higher admission RDW and a previous diagnostic history of diabetes and admission higher serum sodium concentrations. All-cause mortality was significantly higher among patients with RDW  15% at one year of follow-up (29.6% vs. 23.2%, p 0.026). Multivariate analysis confirmed the association between RDW and higher risk of one-year mortality, as well as with older age, higher Charlson comorbidity Index, higher potassium serum concentrations and no hypertension as a previous diagnosis.

Conclusions: In elderly patients experiencing their first admission due to acute HF, a higher RDW at baseline might help identify patients at higher risk for one-year all-cause mortality.

/pdf/red-blood-cell-distribution-width-as-a-prognostic-factor-of-l18893w6vq.pdf

Red blood cell distribution width as a prognostic factor of mortality in elderly patients firstly hospitalized due to heart failure

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Longitudinal changes in serum ferritin levels correlate with measures of hepatic stiffness in transfusion-independent patients with β-thalassemia intermedia

Lymphocyte-to-white blood cells ratio in older patients experiencing a first acute heart failure hospitalization.

Hyponatremia: a challenge for internists.

Red blood cell distribution width as a prognostic factor of mortality in elderly patients firstly hospitalized due to heart failure