Article
Autoimmune lymphoproliferative syndrome-like disease with somatic KRAS mutation.
Blood - 10 Mar 2011
Takagi Masatoshi, Shinoda Kunihiro, Piao Jinhua, Mitsuiki Noriko, Takagi Mari, Matsuda Kazuyuki, Muramatsu Hideki, Doisaki Sayoko, Nagasawa Masayuki, Morio Tomohiro, Kasahara Yoshihito, Koike Kenichi, Kojima Seiji, Takao Akira, Mizutani Shuki
Abstract excerpt
Autoimmune lymphoproliferative syndrome (ALPS) is classically defined as a disease with defective FAS-mediated apoptosis (type I-III). Germline NRAS mutation was recently identified in type IV ALPS. We report 2 cases with ALPS-like disease with somatic KRAS mutation. Both cases were characterized by prominent autoimmune cytopenia and lymphoadenopathy/splenomegaly. These patients did not satisfy the diagnostic...
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