OBSOLETE: Lymphoproliferative syndrome

Lymphoproliferative syndrome is a term that has been used to describe a group of conditions where the immune system produces too many white blood cells called lymphocytes. This entry in Orphanet (code 238510) is now marked as OBSOLETE, meaning it has been retired and replaced by more specific disease classifications. The conditions previously grouped under this name are now categorized under more precise diagnoses such as autoimmune lymphoproliferative syndrome (ALPS), X-linked lymphoproliferative disease (XLP), or other specific lymphoproliferative disorders. In these conditions, the body's lymphocytes grow and multiply in an uncontrolled way, which can lead to enlarged lymph nodes, an enlarged spleen, an enlarged liver, and problems with the immune system. Some forms are inherited and caused by genetic mutations that affect how lymphocytes normally die off when they are no longer needed. Without proper cell death (called apoptosis), these extra immune cells build up and can cause autoimmune problems, where the body attacks its own healthy tissues. Because this is an obsolete classification, patients who were previously diagnosed under this umbrella term should work with their doctors to determine which specific lymphoproliferative condition they have. Treatment varies widely depending on the exact diagnosis but may include immunosuppressive medications, targeted therapies, monitoring for lymphoma, and in some cases bone marrow transplantation.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.Which specific type of lymphoproliferative syndrome do I or my child have?,What genetic testing should be done to confirm the diagnosis?,What is the risk of developing lymphoma, and how will you monitor for it?,What medications are recommended, and what are their side effects?,Is bone marrow transplant an option for my specific condition?,Are other family members at risk and should they be tested?,What symptoms should prompt me to go to the emergency room?

Common questions about OBSOLETE: Lymphoproliferative syndrome