title: Neonatal hemophagocytic lymphohistiocytosis associated with a vertical transmission of coxsackievirus B1.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by fever, cytopenias, hepatosplenomegaly, and coagulopathy with the background of hypercytokinemia. Early diagnosis and etoposide therapy are not established for affected newborns. An afebrile infant suffered from apnea 4 days after birth, showing leukocytosis, thrombocytopenia, coagulopathy, and cerebrospinal fluid pleocytosis. Serum levels of ferritin and sIL-2R were high. Bone marrow studies revealed activated/hemophagocytosing macrophages. Coxsackievirus B1 (CB1) was isolated from the throat and stool. Serum anti-CB1 antibody titers were elevated in the patient (4 → 16; 6 → 43 days after birth) and mother (128; 10 days after delivery). Normal expressions of perforin and CD107a precluded inherited HLH. The vertically transmitted CB1-HLH was successfully treated without administration of corticosteroid, cyclosporine, or etoposide. Serum cytokine levels showed dominant expression of monokines (IL-1β/6/8, and TNF-α) but not IFN-γ, which is the central player of inherited HLH. The cytokine profile might represent a unique pathophysiology of enterovirus-driven neonatal HLH.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi Higashi-ku, Fukuoka, 812-8582, Japan.
authors.forename: Mitsuharu
authors.initials: M
authors.lastname: Fukazawa
authors.forename: Takayuki
authors.initials: T
authors.lastname: Hoshina
authors.forename: Etsuro
authors.initials: E
authors.lastname: Nanishi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Nishio
authors.forename: Takehiko
authors.initials: T
authors.lastname: Doi
authors.forename: Shouichi
authors.initials: S
authors.lastname: Ohga
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D029822:Enterovirus B, Human / Q000302:isolation & purification*; D004769:Enterovirus Infections / Q000635:transmission* / Q000821:virology*; D006801:Humans; D007231:Infant, Newborn; D018445:Infectious Disease Transmission, Vertical; D051359:Lymphohistiocytosis, Hemophagocytic / Q000821:virology*; D008297:Male