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Autoimmune Lymphoproliferative Syndrome 

Autoimmune Lymphoproliferative Syndrome
Chapter:
Autoimmune Lymphoproliferative Syndrome
Author(s):

Thomas A. Fleisher

, Frederic Rieux-Laucat

, and Jennifer M. Puck

DOI:
10.1093/med/9780195389838.003.0030
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date: 12 December 2017

Primary immunodeficiency diseases are inherited disorders that affect human adaptive and innate immunity. In most cases, affected individuals experience recurrent infections, but they may also suffer from autoimmune diseases and malignancies. This chapter focuses on Autoimmune Lymphoproliferative Syndrome (ALPS), including the historic and scientific background, clinical presentations, immunologic characteristics, and the molecular/genetic underpinnings. Where appropriate, diagnostic tools and therapeutic options are outlined -- from prophylactic anti-infective measures to hematopoietic stem cell transplantation and gene therapy.

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