ABSTRACT
Ultrasound is a minimally invasive examination method. Previous examinations have shown that the most common standard methods used to reveal the distal aspect of the congenital labial sinus, fistula probe or methylene blue dye, are highly invasive and ultrasound is less invasive. Of course, there are cases where CT is necessary, but CT carries the risk of radiation exposure.
Subject(s)
Argyria , Laser Therapy , Lasers, Solid-State , Argyria/diagnosis , Argyria/etiology , Humans , Lasers, Solid-State/therapeutic useABSTRACT
BACKGROUND: X-linked lymphoproliferative syndrome (XLP) is a rare inherited immunodeficiency by an extreme vulnerability to Epstein-Barr virus (EBV) infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP are now divided into type 1 (XLP-1) and type 2 (XLP-2), which are caused by mutations of SH2D1A/SLAM-associated protein (SAP) and X-linked inhibitor of apoptosis protein (XIAP) genes, respectively. The diagnosis of XLP in individuals with EBV-associated HLH (EBV-HLH) is generally difficult because they show basically similar symptoms to sporadic EBV-HLH. Although EBV-infected cells in sporadic EBV-HLH are known to be mainly in CD8+ T cells, the cell-type of EBV-infected cells in EBV-HLH seen in XLP patients remains undetermined. METHODS: EBV-infected cells in two patients (XLP-1 and XLP-2) presenting EBV-HLH were evaluated by in EBER-1 in situ hybridization or quantitative PCR methods. RESULTS: Both XLP patients showed that the dominant population of EBV-infected cells was CD19+ B cells, whereas EBV-infected CD8+ T cells were very few. CONCLUSIONS: In XLP-related EBV-HLH, EBV-infected cells appear to be predominantly B cells. B cell directed therapy such as rituximab may be a valuable option in the treatment of EBV-HLH in XLP patients.
