Article
Fatal sibling cases of familial hemophagocytic lymphohistiocytosis (FHL) with MUNC13-4 mutations: case reports.
Pediatric hematology and oncology - 1 Jan 2000
Nakao Tomohei, Shimizu Takashi, Fukushima Takashi, Saito Makoto, Okamoto Miho, Sugiura Masatoshi, Yamamoto Ken, Ueda Ikuyo, Imashuku Shinsaku, Kobayashi Chie, Koike Kazutoshi, Tsuchida Masahiro, Sumazaki Ryo, Matsui Akira
Abstract excerpt
The authors report here sibling cases of familial hemophagocytic lymphohistiocytosis (FHL) type 3 that took fatal courses despite intensive treatment. The older brother achieved remission by immunochemotherapy, but a central nervous system lesion occurred before the introduction of allogeneic hematopoietic stem cell transplantation (allo-HSCT). The patient died on day +1 of allo-HSCT due to progression of the...
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