A Term Infant of Neonatal Toxic Shock Syndrome-Like Exanthematous Disease Complicated with Hemophagocytic Syndrome

Abstract

Neonatal toxic shock syndrome-like exanthematous disease (NTED) is a newly recognized neonatal infectious disease, caused by the superantigen toxic shock syndrome toxin-1 (TSST-1). TSST-1 is mainly produced by methicillin-resistant Staphylococcus aureus, and the immune responses to TSST-1 are known to cause toxic shock syndrome, a life-threatening infectious disease. The clinical symptoms of NTED are skin rash, fever, and thrombocytopenia, but severe thrombocytopenia is rare in term infants with NTED. Although the cause of NTED is the same as that of toxic shock syndrome, the clinical symptoms of NTED are milder than toxic shock syndrome. The mild phenotype of NTED has been explained by selectively elevated serum levels of anti-inflammatory cytokine interleukin (IL)-10, which suppress immune responses to TSST-1. In the present study, we report a term female infant of NTED complicated with hemophagocytic syndrome (HPS). HPS is characterized by systemic inflammation and hemophagocytosis, caused by uncontrolled activation of T cells and macrophages. The serum IL-10 level of the patient at 4 days of age was relatively low (67 pg/mL) for NTED but still higher than normal controls (< 2.0 pg/mL). The patient also showed severe thrombocytopenia. We speculate that the serum IL-10 level of the patient was enough to supress immune responses to TSST-1, thereby resulting in NTED, but not enough to suppress the onset of HPS. This is the first reported case of NTED complicated with HPS. If a physician encounters an NTED patient with severe cytopenia, microscopic examination of peripheral blood smear should be carried out to exclude HPS.

Neonatal hemophagocytic lymphohistiocytosis: A meta‐analysis of 205 cases

“…Studies were excluded if they did not discuss nHLH ( n = 280); aggregated nHLH patient data with all ages ( n = 83); provided insufficient detail and/or data describing a case of nHLH ( n = 28); and for other reasons ( n = 8). Two‐hundred‐five cases of nHLH from 142 articles were included in our analysis 12–152 …”

A Rare Case of Staphylococcal Toxic Shock Syndrome in a Neonate

“…TSS in neonates is relatively a newly recognized neonatal infectious disease, although it was first described by Todd et al in 1978 in previously healthy children [ 13 , 14 ]. There are limited data describing epidemiology, management, and outcomes for children with TSS [ 1 , 15 ].…”

Peripheral platelet phagocytosis in an extremely low birth weight infant:a case report

“…The observation of hemophagocytic macrophages in a peripheral blood smear is uncommon. Kaga et al 6) reported a neonate with neonatal toxic shock syndrome (TSS) -like exanthematous disease (NTED) complicated by hemophagocytic syndrome (HPS). In this case, the patient did not meet the diagnostic criteria of HPS, socalled hemophagocytic lymphohistiocytosis (HLH).…”