Familial Hemophagocytic Lymphohistiocytosis Presenting as Hydrops Fetalis

• Main Authors: Sota Iwatani (Author), Kazuya Uemura (Author), Masami Mizobuchi (Author), Seiji Yoshimoto (Author), Keiichiro Kawasaki (Author), Yoshiyuki Kosaka (Author), Masayuki Hori (Author), Takahiro Yasumi (Author), Hideto Nakao (Author)
• Format: Book
• Published: Thieme Medical Publishers, Inc., 2015-03-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Abstract Background Familial hemophagocytic lymphohistiocytosis (FLH) is an autosomal recessive disorder of immune regulation that leads to a hyperinflammatory syndrome. Fetal onset FHL is extremely rare and is considered to be the most severe form of FHL. Case We report a preterm case of FHL that p...