Purpose: Patients who receive frequent blood transfusions are at an elevated risk of developing hepatic fibrosis due to iron overload in the liver. In this study, we evaluated the effectiveness of transient elastography (TE) (FibroScan® ) for assessing liver fibrosis in patients with pediatric cancer. Methods: We enrolled 106 consecutive cases of acute leukemia in individuals under 21 years of age. The participants were followed for 2 years. Based on their serum ferritin (SF) levels, the patients were divided into two groups: group 1 (SF≥300 ng/mL) and group 2 (SF<300 ng/mL). A liver FibroScan® was performed, and a p-value of less than 0.05 was considered statistically significant. Results: Among the various parameters in the liver function test (LFT), alkaline phosphatase was significantly higher in a subgroup of patients aged 5–8 years in group 2 compared to those in group 1. The indices of liver fibrosis determined by TE, including the FibroScan score, controlled attenuation parameter score, steatosis percentage, and meta-analysis of histological data in viral hepatitis score, as well as indirect serum markers of liver fibrosis such as the aminotransferase (AST)/alanine aminotransferase (ALT) ratio, Fibrosis 4 score, and AST to platelet ratio index, did not differ significantly between the two groups. The association between the TE results and LFT parameters was only significant for ALT. Conclusion Transfusion-associated iron overload does not have a significant correlation with severe liver fibrosis. FibroScan® is not a sensitive tool for detecting early stages of fibrosis in survivors of pediatric leukemia.

Background: Development of antibodies against infused Factor VIII (FVIII) or "inhibitors" represents a major challenge following FVIII replacement therapy in patients with hemophilia A (HA). Recent studies have shown that certain cellular compartments of the immune system contribute to the production of such antibodies. Herein, we determined the frequency of class-switched ­CD19+IgD−CD27+ on-class-switched ­CD19+IgD+CD27+ memory B cell subsets and ­CD19 + CD27hiCD38hi plasmablasts in patients with severe HA and their association with the development of inhibitors in these patients. Methods: This cross-sectional case–control study enrolled 32 patients with severe HA, including 8 with and 24 without inhibitors, and 24 healthy individuals. The frequencies of the memory B cell subsets and plasmablasts were determined using flow cytometry. Results: The frequency of ­­CD19+IgD+CD27+ non-class-switched memory B cells was significantly lower in patients with HA (including both patients with and without inhibitors) than in healthy controls. The percentages of both ­CD19+IgD−CD27+ class-switched and ­­CD19+IgD+CD27+ non-class-switched memory B cells did not differ significantly between patients with and without inhibitors. HA patients with inhibitors had significantly higher proportions of ­CD19+CD27hiCD38hi plasmablasts than the control group as well as the inhibitor (-) ones. No significant correlation was observed between the inhibitor levels with the percentages of memory B cell subsets and plasmablasts. Conclusion This study is the first to demonstrate a dysregulated proportion of ­CD19+IgD+CD27+ non-class-switched memory B cells and ­CD19+CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.

Background: Development of antibodies against infused Factor VIII (FVIII) or "inhibitors" represents a major challenge following FVIII replacement therapy in patients with hemophilia A (HA). Recent studies have shown that certain cellular compartments of the immune system contribute to the production of such antibodies. Herein, we determined the frequency of class-switched ­CD19+IgD−CD27+ on-class-switched ­CD19+IgD+CD27+ memory B cell subsets and ­CD19 + CD27hiCD38hi plasmablasts in patients with severe HA and their association with the development of inhibitors in these patients. Methods: This cross-sectional case–control study enrolled 32 patients with severe HA, including 8 with and 24 without inhibitors, and 24 healthy individuals. The frequencies of the memory B cell subsets and plasmablasts were determined using flow cytometry. Results: The frequency of ­­CD19+IgD+CD27+ non-class-switched memory B cells was significantly lower in patients with HA (including both patients with and without inhibitors) than in healthy controls. The percentages of both ­CD19+IgD−CD27+ class-switched and ­­CD19+IgD+CD27+ non-class-switched memory B cells did not differ significantly between patients with and without inhibitors. HA patients with inhibitors had significantly higher proportions of ­CD19+CD27hiCD38hi plasmablasts than the control group as well as the inhibitor (-) ones. No significant correlation was observed between the inhibitor levels with the percentages of memory B cell subsets and plasmablasts. Conclusion This study is the first to demonstrate a dysregulated proportion of ­CD19+IgD+CD27+ non-class-switched memory B cells and ­CD19+CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.

Background: Development of antibodies against infused Factor VIII (FVIII) or "inhibitors" represents a major challenge following FVIII replacement therapy in patients with hemophilia A (HA). Recent studies have shown that certain cellular compartments of the immune system contribute to the production of such antibodies. Herein, we determined the frequency of class-switched ­CD19+IgD−CD27+ on-class-switched ­CD19+IgD+CD27+ memory B cell subsets and ­CD19 + CD27hiCD38hi plasmablasts in patients with severe HA and their association with the development of inhibitors in these patients. Methods: This cross-sectional case–control study enrolled 32 patients with severe HA, including 8 with and 24 without inhibitors, and 24 healthy individuals. The frequencies of the memory B cell subsets and plasmablasts were determined using flow cytometry. Results: The frequency of ­­CD19+IgD+CD27+ non-class-switched memory B cells was significantly lower in patients with HA (including both patients with and without inhibitors) than in healthy controls. The percentages of both ­CD19+IgD−CD27+ class-switched and ­­CD19+IgD+CD27+ non-class-switched memory B cells did not differ significantly between patients with and without inhibitors. HA patients with inhibitors had significantly higher proportions of ­CD19+CD27hiCD38hi plasmablasts than the control group as well as the inhibitor (-) ones. No significant correlation was observed between the inhibitor levels with the percentages of memory B cell subsets and plasmablasts. Conclusion This study is the first to demonstrate a dysregulated proportion of ­CD19+IgD+CD27+ non-class-switched memory B cells and ­CD19+CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.

OBJECTIVEComplementary and alternative medicine (CAM) use has an increasing trend around the world. Despite the wild application of CAM in patients with coagulation disorders, its efficacy is still questioned by many studies. The aim of this study was to determine the frequency and types of CAM usage, and the factors affecting CAM use among patients with bleeding disorders.

METHODSThis cross-sectional study investigated the usage of CAM with a standard validated questionnaire in coagulopathic patients who were referred to Dastgheib Hospital, an educational therapeutic center affiliated to the Shiraz University of Medical Sciences in Shiraz, Southern Iran.

RESULTSBetween December 2015 and May 2016, 75 patients were included in this survey. Severe hemophilia A and rare bleeding disorders were the most frequent among our participants. Overall, nearly half of the studied population (49.3%) used at least one CAM product or practices. The most common products were multivitamin (29.3%), followed by folic acid (21.3%) and chamomile (12%). 32% of the patients resorted to faith healing. The main reasons of using CAM were reported to be increased general health, correction of anemia and thrombocytopenia and control of bleeding tendency.

CONCLUSIONCAM is being used frequently in patients with coagulation disorders like many other chronic illnesses all around the world. Due to emerging concern regarding the safety and possible interaction with the conventional medicine, it is necessary to develop basic data about the hematologic effects of these methods in order to use them more safely.