There are 2 types of XLP: XLP-1 and XLP-2. People with XLP-1 develop FIM/HLH after Epstein-Barr virus infection. Normally, the Epstein-Barr virus causes a person to develop mononucleosis, or “mono.” Mono is common among children and teens. A person with mono has a sore throat, swollen glands, fever, and tiredness.

What is X-linked lymphoproliferative (XLP) syndrome? X-linked lymphoproliferative syndrome (XLP), or Duncan’s syndrome, is a rare genetic condition where the immune system does not work properly.

Apr 1, 2019 · People with XLP are healthy until they are exposed to EBV. Then, they can become seriously ill and experience swollen lymph nodes, an enlarged liver and spleen, hepatitis and lymphoma. Some people with XLP have developed lymphoma in the absence of EBV infection.

X-linked lymphoproliferative syndrome (XLP) is a rare genetic disorder that weakens the immune system, making it difficult for the body to fight off certain viral infections, particularly the Epstein-Barr virus (EBV). First identified in the 1970s, XLP is caused by mutations in the SH2D1A or XIAP genes, both located on the X chromosome.

Feb 27, 2004 · X-linked lymphoproliferative disease (XLP) in general is characterized by an inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis, dysgammaglobulinemia, and lymphoproliferative disease (malignant lymphoma). The condition primarily affects males.

People with XLP may respond to EBV infection by producing abnormally large numbers of T cells, B cells, and other lymphocytes called macrophages. This proliferation of immune cells often causes a life-threatening reaction called hemophagocytic lymphohistiocytosis.

May 21, 2019 · Chronic active EBV infection (CAEBV) and EBV-hemophagocytic lymphohistiocytosis (HLH) are recognized as systemic EBV-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (LPDs) arising from the clonal proliferations of EBV-infected T cells and NK cells.

Apr 4, 2018 · Here, we describe clinical features of XLP1, how molecular and biological studies of the gene product, SAP, and the associated signaling lymphocyte activation molecule family receptors have provided insight into disease pathogenesis including specific immune cell defects, and current therapeutic approaches including the potential use of gene the...

The Journal of Leukocyte Biology (JLB) is an international, high-impact journal of the Society for Leukocyte Biology (SLB) dedicated to publishing

Jul 17, 2021 · Xiao Huoluo Pills (XHLP) has been used to treat degenerative diseases such as osteoarthritis and hyperosteogeny. However, XHLP's specific effective ingredients and mechanism of action against osteoarthritis have not been explored.