Autoimmune lymphoproliferative syndrome

Autoimmune lymphoproliferative syndrome
Other namesCanale-Smith syndrome,[1]
SpecialtyImmunology 

Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis.[2]

It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis.[3] Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T lymphocytes and Fas-ligand on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell apoptosis. Patients with ALPS have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, autoimmune disease, and secondary cancers.[4]

  1. ^ Straus SE, Jaffe ES, Puck JM; et al. (Jul 2001). "The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis". Blood. 98 (1): 194–200. doi:10.1182/blood.v98.1.194. PMID 11418480.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ Cite error: The named reference pmid18193364 was invoked but never defined (see the help page).
  3. ^ Cite error: The named reference pmid16522544 was invoked but never defined (see the help page).
  4. ^ Cite error: The named reference pmid19930184 was invoked but never defined (see the help page).
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