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ALPS

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Definition

Autoimmune lymphoproliferative syndrome.ALPS is caused by genetic mutation most often in the FAS gene. It is characterized by non-malignant proliferations of lymphoid tissue resulting in enlargement of lymph nodes and liver and sometimes the spleen- which can result in hypersplenism- a condition in which a large spleen too actively filters the blood, lowering circulating numbers of cells. The condition usually manifests in the first year of life, waxes and wanes, then usually spontaneously improves in the second decade of life. ALPS can be complicated by autoimmune disorders usually affecting mostly the blood cells. The condition is associated with an increased lifetime increased risk of both Hodgkin's and non-Hodgkin's lymphoma. Severe manifestations of the disease may respond to cortisone drugs and other immune suppressive medications. Splenectomy does not cure the condition and results in an increased risk of infections. Bone marrow transplant can be curative but is usually reserved for those with lymphomas or severe subtypes of the disease caused by unusual mutations.


 

 

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