Neonatal hemophagocytic lymphohistiocytosis associated with a vertical transmission of coxsackievirus B1

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

Research output: Contribution to journalArticle

3 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1210-1213
Number of pages4
JournalJournal of Infection and Chemotherapy
Volume19
Issue number6
DOIs
Publication statusPublished - Jan 1 2013

Fingerprint

Hemophagocytic Lymphohistiocytosis
Enterovirus
Leukocytosis
Etoposide
Serum
Parturition
Monokines
Cytokines
Perforin
Apnea
Ferritins
Pharynx
Interleukin-1
Thrombocytopenia
Cyclosporine
Cerebrospinal Fluid
Early Diagnosis
Interleukin-6
Adrenal Cortex Hormones
Fever

All Science Journal Classification (ASJC) codes

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

Cite this

Neonatal hemophagocytic lymphohistiocytosis associated with a vertical transmission of coxsackievirus B1. / Fukazawa, Mitsuharu; Hoshina, Takayuki; Nanishi, Etsuro; Nishio, Hisanori; Doi, Takehiko; Ohga, Shoichi; Hara, Toshiro.

In: Journal of Infection and Chemotherapy, Vol. 19, No. 6, 01.01.2013, p. 1210-1213.

Research output: Contribution to journalArticle

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