- Evans Syndrome After Successful Immunosuppressant-Free Living-Donor Liver Transplant. [Journal Article]
- ECExp Clin Transplant 2018 Jun 18
- Evans syndrome is an uncommon disease characterized by a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia concomitantly or sequentially with a positive direct Coombs test in...
Evans syndrome is an uncommon disease characterized by a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia concomitantly or sequentially with a positive direct Coombs test in the absence of any underlying known cause. Here, we present a case of an adult patient who underwent living-donor liver transplant that was preceded by bone marrow transplant 20 years earlier from the same HLA identical donor and who received a single-agent immunosuppressive therapy for only 2 months as prophylaxis against graft-versus-host disease. Two months after transplant, he developed Evans syndrome with severe anemia and thrombocytopenia. After administration of steroids and intravenous immunoglobulin, the patient's anemia and thrombocytopenia improved dramatically. Through the 7 years of follow-up, the patient has not developed graft-versus-host disease or acute or chronic rejection. This case demonstrates a rare complication posttransplant and the possibility of functional tolerance of liver grafts after a combined liver and bone marrow transplant from the same donor.
- Cytomegalovirus reactivation can be a cause of DAT-negative hemolytic anemia in patients with chronic lymphocytic leukemia. [Journal Article]
- LLLeuk Lymphoma 2018 Jun 18; :1-3
- Squeezing for Life - Properties of Red Blood Cell Deformability. [Review]
- FPFront Physiol 2018; 9:656
- Deformability is an essential feature of blood cells (RBCs) that enables them to travel through even the smallest capillaries of the human body. Deformability is a function of (i) structural elements...
Deformability is an essential feature of blood cells (RBCs) that enables them to travel through even the smallest capillaries of the human body. Deformability is a function of (i) structural elements of cytoskeletal proteins, (ii) processes controlling intracellular ion and water handling and (iii) membrane surface-to-volume ratio. All these factors may be altered in various forms of hereditary hemolytic anemia, such as sickle cell disease, thalassemia, hereditary spherocytosis and hereditary xerocytosis. Although mutations are known as the primary causes of these congenital anemias, little is known about the resulting secondary processes that affect RBC deformability (such as secondary changes in RBC hydration, membrane protein phosphorylation, and RBC vesiculation). These secondary processes could, however, play an important role in the premature removal of the aberrant RBCs by the spleen. Altered RBC deformability could contribute to disease pathophysiology in various disorders of the RBC. Here we review the current knowledge on RBC deformability in different forms of hereditary hemolytic anemia and describe secondary mechanisms involved in RBC deformability.
- A Case Report of Congenital Non-spherocytic Hemolytic Anemia in a Patient from India. [Journal Article]
- CCureus 2018 Apr 13; 10(4):e2478
- Congenital non-spherocytic hemolytic anemia (CNSHA) is a rare autosomal recessive condition that presents as a congenital hemolytic anemia. The absence of vital enzymes required for glycolysis such a...
Congenital non-spherocytic hemolytic anemia (CNSHA) is a rare autosomal recessive condition that presents as a congenital hemolytic anemia. The absence of vital enzymes required for glycolysis such as homozygous glucose phosphate isomerase (GPI) and red blood cell (RBC) nucleotide metabolism predisposes the RBCs to hemolysis. No spherocytosis is seen on peripheral smear as well as no signs of immune-mediated destruction of RBCs. We present a rare case of a previously healthy 21-year-old female patient with CNSHA from India.
- Postponing Early intrauterine Transfusion with Intravenous immunoglobulin Treatment; the PETIT study on severe hemolytic disease of the fetus and newborn. [Journal Article]
- AJAm J Obstet Gynecol 2018 Jun 11
- CONCLUSIONS: Intravenous immunoglobulin treatment in mothers pregnant with a fetus at risk for hemolytic disease seems to have a potential clinically relevant, beneficial effect on the course and severity of the disease. Confirmation in a multicenter randomized trial is needed.
- Outbreak of E. coli O157:H7 Infections Associated with Exposure to Animal Manure in a Rural Community - Arizona and Utah, June-July 2017. [Journal Article]
- MMMMWR Morb Mortal Wkly Rep 2018 Jun 15; 67(23):659-662
- On June 26, 2017, a hospital in southern Utah notified the Utah Department of Health of Shiga toxin-producing Escherichia coli (STEC) O157:H7 infections in two children from a small community on the ...
On June 26, 2017, a hospital in southern Utah notified the Utah Department of Health of Shiga toxin-producing Escherichia coli (STEC) O157:H7 infections in two children from a small community on the Arizona-Utah border. Both children developed hemolytic uremic syndrome, characterized by hemolytic anemia, acute kidney failure, and thrombocytopenia and died within a few days of illness onset. Over the next few days, several more STEC-associated illnesses were reported in residents of the community. A joint investigation by local and state health agencies from Arizona and Utah and CDC was initiated to identify the outbreak source and prevent additional cases; a total of 12 cases were identified, including the two children who died. Investigators initially explored multiple potential sources of illness; epidemiologic and environmental information revealed cow manure contact as the likely initial cause of the outbreak, which was followed by subsequent person-to-person transmission. One of the outbreak strains was isolated from bull and horse manure collected from a yard near a community household with two ill children. Local health agencies made recommendations to the public related to both animal contact and hand hygiene to reduce the risk for STEC transmission. Animal or animal manure contact should be considered a potential source of STEC O157:H7 during outbreaks in communities where ruminants are kept near the home.
- Unchanged Erythrocyte Profile After Exposure to Cryogenic Temperatures in Elder Marathon Runners. [Journal Article]
- FPFront Physiol 2018; 9:659
- Objective: Endurance runners may experience "sports anemia" resulting from intravascular hemolysis. In addition, aging has negative impact on hematopoiesis and rheological properties of blood, and e...
Objective: Endurance runners may experience "sports anemia" resulting from intravascular hemolysis. In addition, aging has negative impact on hematopoiesis and rheological properties of blood, and erythrocyte membranes in older people are more vulnerable to oxidative damage, which together can lead to anemia. Whole-body cryostimulation (WBCST) is increasingly used in the elderly as a method of biological regeneration of athletes or therapy and preventive treatment. That is why the aim of the study was to determine whether repeated WBCST had an effect on the erythrocyte system in master marathon runners, compared to non-training men. Methods: Ten marathon runners (men aged 55.9 ± 5.5 years, training experience 6.71 ± 5.79 years) and 10 non-training (men aged 62.0 ± 5.8 years) were subjected to a series of 24 WBCST (3 min, -130°C) performed every other day. Erythrocyte levels, interleukin-3 (IL-3), erythropoietin (EPO), haptoglobin, bilirubin, and extracellular hemoglobin (HGBecf) concentrations were determined in the blood before and after 12, 24 WBCST, as well as 7 days after their completion. Results: The concentrations of EPO and IL-3 were significantly increased 7 days after the completion of WBCST in both groups (P < 0.05). The erythrocyte content and indicators, the bilirubin, haptoglobin, and HGBecf levels in each group did not change as a result of WBCST. In order to document hemolytic changes and/or factors affecting the severity of erythropoiesis, correlations between growth erythropoietic factors, erythrocyte and hemolytic factors as well as mutual correlations between hemolytic indexes were calculated. There was a positive correlation (P < 0.05) between the EPO and IL-3, bilirubin, mean corpuscular hemoglobin, and red blood cell distribution width - standard deviation. There was also a positive correlation between the concentrations of bilirubin and HGBecf, and a negative correlation between haptoglobin and HGBecf as well as bilirubin concentrations. Conclusion: WBCST treatments, repeated every other day, do not cause hemolytic changes in elder men with high or low physical activity. But also, they are a procedure that does not increase the level of erythrocytes or their hemoglobinization. In athletes, it is not a form of doping. The positive correlation between EPO and bilirubin may be indicative of, for example, the mutual antioxidative effect of these factors.
- Disseminated cryptococcosis associated with administration of eculizumab. [Journal Article]
- AJAm J Health Syst Pharm 2018 Jun 12
- CONCLUSIONS: A 23-year-old man with a history of minimal change nephrotic syndrome was hospitalized for acute kidney injury and abdominal pain and swelling. He was found to have disseminated pneumococcal disease, including peritonitis, bacteremia, and pulmonic endocarditis. The patient developed evidence of microangiopathic hemolytic anemia, leading to a diagnosis of atypical hemolytic uremic syndrome, and was started on eculizumab. The patient initially improved but developed septic shock 18 days after the first dose of eculizumab. All subsequent blood, respiratory, and intraabdominal cultures grew Cryptococcus neoformans. Twenty-eight days after the initial dose of eculizumab, the patient died. Autopsy findings demonstrated disseminated cryptococcosis, with infection noted in lung, myocardial, kidney, and liver tissues. Considering the complement-dependent nature of host defenses against Cryptococcus species and available evidence regarding C. neoformans pathogenicity from studies of murine models, it was hypothesized that the patient's cryptococcosis and death were secondary to administration of eculizumab and consequent blockage of the C5 component of the complement cascade. Eculizumab is known to predispose recipients to infections with encapsulated organisms; however, this was believed to be the first reported case of infection with an encapsulated yeast possibly due to eculizumab use. Patients receiving eculizumab should be monitored closely for invasive cryptococcal infections.A 23-year-old man developed fatal disseminated cryptococcosis after treatment with eculizumab.
- Biocomputing for portable, resettable and quantitative point-of-care diagnostics: making glucose meter a logic-gate responsive device to many targets in clinical care. [Journal Article]
- ACAngew Chem Int Ed Engl 2018 Jun 12
- While biocomputing is recognized to provide intelligent solution to complex biosensing projects, it remains challenging to transform biomolecular logic gates into a convenient, portable, resettable a...
While biocomputing is recognized to provide intelligent solution to complex biosensing projects, it remains challenging to transform biomolecular logic gates into a convenient, portable, resettable and quantitative sensing system for point-of-care (POC) diagnostics in a low-resource setting. To overcome these limitations, we herein report the first design and demonstration of biocomputing on personal glucose meters (PGM) by utilizing glucose and nicotinamide adenine dinucleotide as signal outputs, DNAzyme and protein enzymes as building blocks and demonstrating a general platform for installing logic-gate responses (YES, NOT, INHIBIT and OR) to a variety of biological species, such as cations (sodium), anions (citrate), organic metabolites (ADP, ATP) and enzymes (pyruvate kinase, alkaline phosphatase, alcohol dehydrogenases). A concatenated logical gate platform that is resettable is also demonstrated. Our system is highly modular and can be generally applied to POC diagnostics of many diseases such as hyponatremia, hypernatremia and hemolytic anemia. In addition to making PGM even wider in its clinical applications, the method reported here open a new avenue in biomolecular logic gates for the developing intelligent POC devices for on-site applications.
New Search Next
- Pediatric thrombotic thrombocytopenic purpura. [Review]
- EJEur J Haematol 2018 Jun 11
- Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (...
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation. Consequently, microangiopathic hemolytic anemia and consumption thrombocytopenia are associated with organ ischemia. The differential diagnosis with other TMAs, autoimmune cytopenias or hematological malignancies may be challenging. The exploration of ADAMTS13 (activity, antibodies, antigen, ADAMTS13 gene) supports the diagnosis of TTP. The first-line treatment of the acute phase of TTP is based on plasmatherapy. In congenital TTP, patients with a chronic disease benefit from a prophylactic plasmatherapy. In autoimmune TTP, steroids and B-cells depleting therapies increasingly are used together with plasma exchange. Long-term follow-up including the monitoring of ADAMTS13 activity is mandatory. A severe decrease in ADAMTS13 activity (< 10%) may predict relapses and preemptive B-cell depletion with rituximab can be used to prevent relapses. This article is protected by copyright. All rights reserved.