Neonatal hemophagocytic lymphohistiocytosis associated with a vertical transmission of coxsackievirus B1

Mitsuharu Fukazawa, Takayuki Hoshina, Etsuro Nanishi, Hisanori Nishio, Takehiko Doi, Shouichi Ohga, Toshiro Hara

Research output: Contribution to journalArticlepeer-review

9 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1210-1213
Number of pages4
JournalJournal of Infection and Chemotherapy
Issue number6
Publication statusPublished - Dec 2013

All Science Journal Classification (ASJC) codes

  • Microbiology (medical)
  • Infectious Diseases
  • Pharmacology (medical)


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