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 language | English |
|---|---|
| Pages (from-to) | 1210-1213 |
| Number of pages | 4 |
| Journal | Journal of Infection and Chemotherapy |
| Volume | 19 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Dec 2013 |
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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, Shouichi; Hara, Toshiro.
In: Journal of Infection and Chemotherapy, Vol. 19, No. 6, 12.2013, p. 1210-1213.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Neonatal hemophagocytic lymphohistiocytosis associated with a vertical transmission of coxsackievirus B1
AU - Fukazawa, Mitsuharu
AU - Hoshina, Takayuki
AU - Nanishi, Etsuro
AU - Nishio, Hisanori
AU - Doi, Takehiko
AU - Ohga, Shouichi
AU - Hara, Toshiro
PY - 2013/12
Y1 - 2013/12
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84890859301&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84890859301&partnerID=8YFLogxK
U2 - 10.1007/s10156-013-0629-2
DO - 10.1007/s10156-013-0629-2
M3 - Article
C2 - 23757031
AN - SCOPUS:84890859301
VL - 19
SP - 1210
EP - 1213
JO - Journal of Infection and Chemotherapy
JF - Journal of Infection and Chemotherapy
SN - 1341-321X
IS - 6
ER -