Learn About X-Linked Lymphoproliferative Disease

What is the definition of 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.

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What are the causes of X-Linked Lymphoproliferative Disease?

Mutations in the SH2D1A and XIAP genes cause XLP. SH2D1A gene mutations cause XLP1, and XIAP gene mutations cause XLP2.

How prevalent is X-Linked Lymphoproliferative Disease?

XLP1 is estimated to occur in about 1 per million males worldwide. XLP2 is less common, occurring in about 1 per 5 million males.

Is X-Linked Lymphoproliferative Disease an inherited disorder?

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.

Who are the top X-Linked Lymphoproliferative Disease Local Doctors?
Elite
Highly rated in
13
conditions
Pediatrics
Pediatric Hematology Oncology

Cincinnati Childrens Hospital Medical Center

Burnet Campus

3333 Burnet Ave 
Cincinnati, OH 45229

Rebecca Marsh is a Pediatrics specialist and a Pediatric Hematologist Oncology doctor in Cincinnati, Ohio. Dr. Marsh has been practicing medicine for over 19 years and is rated as an Elite doctor by MediFind in the treatment of X-Linked Lymphoproliferative Disease. She is also highly rated in 13 other conditions, according to our data. Her top areas of expertise are X-Linked Lymphoproliferative Disease, Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, and Histiocytosis. She is licensed to treat patients in Ohio. Dr. Marsh is currently accepting new patients.

Elite
Highly rated in
6
conditions

Baqiyatallah Research Center For Gastroenterology & Liver Disease, Baqiyatallah University Of Medical Sciences

Baqiyatallah Research Center For Gastroenterology And Liver Disease 
Tehran, IR 

Hossein Khedmat is in Tehran, Islamic Republic of Iran. Khedmat is rated as an Elite expert by MediFind in the treatment of X-Linked Lymphoproliferative Disease. He is also highly rated in 6 other conditions, according to our data. His top areas of expertise are X-Linked Lymphoproliferative Disease, Kidney Transplant, Helicobacter Pylori Infection, and Metabolic Syndrome.

 
 
 
 
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Elite
Highly rated in
10
conditions

University Of Leuven

Leuven, VLG, BE 

Daan Dierickx is in Leuven, Belgium. Dierickx is rated as an Elite expert by MediFind in the treatment of X-Linked Lymphoproliferative Disease. He is also highly rated in 10 other conditions, according to our data. His top areas of expertise are X-Linked Lymphoproliferative Disease, Autoimmune Hemolytic Anemia, Bone Marrow Transplant, and Mononucleosis.

What are the latest X-Linked Lymphoproliferative Disease Clinical Trials?
Safety and Efficacy of CD19 and CD22 Targeted CAR-T Therapy for Relapsed/Refractory B Cell Leukemia and Lymphoma
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A Phase II, Single-arm Trail of Chidamide Combined With Rituximab and High-dose Methotrexate in Previously Untreated Patients With Primary Central Nervous System Lymphoma
Who are the sources who wrote this article ?

Published Date: May 17, 2021Published By: National Institutes of Health

What are the Latest Advances for X-Linked Lymphoproliferative Disease?
Unrelated cord blood transplantation for adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders.
Reduction of immunosuppression combined with whole-brain radiotherapy and concurrent systemic rituximab is an effective yet toxic treatment of primary central nervous system post-transplant lymphoproliferative disorder (pCNS-PTLD): 14 cases from the prospective German PTLD registry.
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Post-transplant lymphoproliferative disorder localized to the colon presented with mild diarrhea after cord blood transplantation.