Article
Human CTL-based functional analysis shows the reliability of a munc13-4 protein expression assay for FHL3 diagnosis.
Blood - 3 May 2018
Shibata Hirofumi, Yasumi Takahiro, Shimodera Saeko, Hiejima Eitaro, Izawa Kazushi, Kawai Tomoki, Shirakawa Ryutaro, Wada Taizo, Nishikomori Ryuta, Horiuchi Hisanori, Ohara Osamu, Ishii Eiichi, Heike Toshio
Abstract excerpt
Familial hemophagocytic lymphohistiocytosis (FHL) is the major form of hereditary hemophagocytic lymphohistiocytosis (HLH); as such, it requires prompt and accurate diagnosis. We previously reported that FHL type 3 (FHL3) can be rapidly screened by detecting munc13-4 expression in platelets using flow cytometry; however, the reliability of the munc13-4 expression assay for FHL3 diagnosis is unclear. Regardless of...
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