X-Linked Lymphoproliferative DiseaseSymptoms, Doctors, Treatments, Advances & More
X-Linked Lymphoproliferative Disease Overview
Learn About X-Linked Lymphoproliferative Disease
X-linked lymphoproliferative disease (XLP) is a disorder of the immune system and blood-forming cells that is found almost exclusively in males. More than half of individuals with this disorder experience an exaggerated immune response to the Epstein-Barr virus (EBV). EBV is a very common virus that eventually infects most humans. In some people it causes infectious mononucleosis (commonly known as "mono"). Normally, after initial infection, EBV remains in certain immune system cells (lymphocytes) called B cells. However, the virus is generally inactive (latent) because it is controlled by other lymphocytes called T cells that specifically target EBV-infected B cells.
Mutations in the SH2D1A and XIAP genes cause XLP. SH2D1A gene mutations cause XLP1, and XIAP gene mutations cause XLP2.
XLP1 is estimated to occur in about 1 per million males worldwide. XLP2 is less common, occurring in about 1 per 5 million males.
This condition is generally inherited in an X-linked recessive pattern. The genes associated with this condition are located on the X chromosome, which is one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of an associated gene in each cell is sufficient to cause the condition. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons.
Great Ormond Street Hospital For Sick Children NHS Foundation Trust
Claire Booth practices practicing medicine in London, United Kingdom. Ms. Booth is rated as an Elite expert by MediFind in the treatment of X-Linked Lymphoproliferative Disease. She is also highly rated in 15 other conditions, according to our data. Her clinical expertise encompasses X-Linked Lymphoproliferative Disease, Severe Combined Immunodeficiency (SCID), Adenosine Deaminase Deficiency, Leukocyte Adhesion Deficiency Type 1, and Bone Marrow Transplant.
Hirokazu Kanegane practices practicing medicine in Tokyo, Japan. Kanegane is rated as an Elite expert by MediFind in the treatment of X-Linked Lymphoproliferative Disease. They are also highly rated in 33 other conditions, according to our data. Their clinical expertise encompasses X-Linked Lymphoproliferative Disease, Primary Immunodeficiency (PID), IPEX Syndrome, X-Linked Agammaglobulinemia, and Bone Marrow Transplant.
Cincinnati Children's Hospital Medical Center
Rebecca Marsh is a Pediatrics provider practicing medicine in Cincinnati, Ohio. Dr. Marsh is rated as an Elite provider by MediFind in the treatment of X-Linked Lymphoproliferative Disease. She is also highly rated in 16 other conditions, according to our data. Her clinical expertise encompasses Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Histiocytosis, Reticulohistiocytoma, and Bone Marrow Transplant.
Published Date: May 17, 2021
Published By: National Institutes of Health