Evans Syndrome

Evans syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous presence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). At our company, we are committed to making a difference in the field of Evans syndrome by providing comprehensive diagnostics, driving therapy development, establishing reliable animal models, and conducting essential preclinical research.

Overview of Evans Syndrome

Evans syndrome is a complex autoimmune disorder characterized by the immune system mistakenly attacking the body's own red blood cells (AIHA) and platelets (ITP). This results in the destruction of red blood cells and a decrease in platelet count. The development of Evans syndrome is associated with immune system dysregulation, leading to the production of autoantibodies that specifically target red blood cells and platelets. This dysregulation can be triggered by various factors, including viral infections, genetic mutations, and abnormalities in immune cell signaling pathways.

Extensive research has indicated that B cells play a crucial role in the pathogenesis of Evans syndrome. These cells are responsible for producing autoantibodies that bind to red blood cells and platelets, leading to their destruction. Additionally, dysfunctional T cells and impaired regulatory T cell function contribute to the progression of Evans syndrome.

Evans syndrome with acute kidney injury.Fig.1 Light microscopy and electron microscopy analysis of Evans syndrome. (Lin H., et al., 2019)

Drug Discovery and Development for Evans Syndrome

Corticosteroids, such as prednisone, are commonly employed as the initial therapeutics for Evans syndrome. Their mechanism of action involves immune system suppression and reduction in autoantibody production. Intravenous immunoglobulin (IVIg) is an alternative therapeutic option for Evans syndrome. IVIg contains a concentrated amount of antibodies that can modulate the immune response and decrease autoantibody production. Furthermore, immunosuppressive agents like azathioprine and mycophenolate mofetil are frequently utilized as steroid-sparing agents for the long-term management of Evans syndrome. These medications function by suppressing the activity of immune cells involved in autoantibody production.

Our company is committed to developing innovative therapies for Evans syndrome. Through extensive research and collaborations, we strive to identify novel drug targets and therapeutic approaches. You can click on the links below to learn about the therapeutic development solutions you are looking for.

Our Services

Our diagnostic services utilize state-of-the-art techniques to accurately diagnose Evans syndrome. We employ comprehensive laboratory testing, including complete blood counts, antibody testing, and flow cytometry, to identify the presence of AIHA and ITP. Our experienced team of experts provides accurate and professional diagnostics development services, resulting in personalized therapy development solutions.

CTLA-4/LRBA Genetic Engineering Models

CTLA-4 is an inhibitory receptor found on regulatory T-cells, and its deficiency disrupts immune homeostasis. LRBA, an intracellular protein, binds to CTLA-4, preventing its degradation and further contributing to immune dysregulation. By utilizing gene editing technologies such as CRISPR-Cas9, we can precisely modify the CTLA-4 and LRBA gene.

CTLA-4/LRBA Genetic Engineering Models

Tripeptidyl peptidase 2 (TPP2) has recently gained attention as a key molecule in aging, immunosenescence, autoimmunity, and tumorigenesis. Its deficiency has been linked to ES, and serological analysis has shown associations between TPP2 deficit and the presence of specific antibodies. We can genetically engineer TPP2 in animals to meet your experimental needs.

Utilizing primary cells or cell lines, we simulate the complex interactions between immune cells, erythrocytes, and platelets. By examining the cytokine profiles, cellular phenotypes, and signaling pathways, we gain insights into the immune dysregulation underlying ES pathogenesis.

By generating organoids derived from patient-specific stem cells, we can replicate the microenvironment and cellular composition of these tissues. This allows researchers to investigate the effects of various factors, such as immune cell infiltration, cytokine signaling, and the impact of genetic alterations, on disease development and progression.

Our preclinical research services center around performing meticulous and thorough studies to assess the safety and effectiveness of potential therapeutic interventions for Evans syndrome. Along with the aforementioned services and models, we also offer tailored services and the development of disease models to cater to your specific requirements. If you have an interest in our services, please feel free to contact us without any hesitation.

References

  1. Lin H., et al. "Evans syndrome with acute kidney injury." Archives of Iranian Medicine 22.6 (2019): 336.
  2. Audia S., et al. "Evans' syndrome: from diagnosis to treatment." Journal of Clinical Medicine 9.12 (2020): 3851.
For research use only. Not intended for any clinical use.