Virus-associated haemophagocytic syndrome (VAHS) is a non-neoplastic, generalized histiocytic proliferation disorder showing marked haemophagocytosis associated with systemic viral infection. We describe the case of a 1-year-old girl with Epstein-Barr virus (EBV)-related VAHS, in whom Southern blot analysis showed monoclonal proliferation of bone marrow cells with the EBV genome; detected with the Xho-1 fragment of the latent infection membrane protein genome. EBV serology showed anti-Epstein-Barr virus nuclear associated antigen (EBNA), anti-viral capsid antigen (VCA)-IgG, anti-VCA-IgA elevation and positive EBNA of Sheep red blood cells (SRBC)-rosette-forming bone marrow cells in the late period of her clinical course, indicative of EBV infection. DNA analysis of her bone marrow cells showed monoclonal rearrangement of the T-cell receptor-beta and -gamma chain genes but not of the immunoglobulin heavy chain genes. Those results suggest that EBV may infect T-cells, after which the cells proliferate monoclonally. Repeated administration of epipodophyllotoxin VP-16-213 induced remission, but adrenocortical steroid, vincristine, and cyclophosphamide had no effect on the patient's condition. Ours is a first case report of VAHS showing monoclonal proliferation of EBV-infected T-cells.
Background/Aims: Tacrolimus (Tac) is an immunosuppressant that is widely used to prevent allograft rejection in patients after liver transplantation. Recently, a Chinese herbal medicine known as Wuzhi Capsule (WZC) was shown to increase Tac blood concentrations by inhibiting the activity of CYP3A5 in animal studies in rats. To date, it remains unexplored whether WZC can be used to reduce the dose requirement for Tac in liver transplant patients with different donor-recipient CYP3A5 genotypes. Materials and Methods: A total of 185 liver transplant patients were enrolled and were divided into four groups according to the combinations of donor-recipient CYP3A5 phenotypes. WZC was given to patients who had C0/D of Tac ≤ 1 ng/ml per mg and required a dose of Tac ≥ 4 mg. Results: The R+/D+ group had the lowest C0, C0/D, and C0/D/W among the four groups. Furthermore, a larger proportion of patients in the CYP3A5 expression groups required Tac dose adjustment to achieve a therapeutic effect and were given Tac with WZC. Notably, the use of WZC significantly increased the blood concentrations of Tac in the CYP3A5 expression groups. Conclusion: WZC significantly increased the C0, C0/D, and C0/D/W in the CYP3A5 expression groups.
The results indicate that RSV-sensitized lymphocytes from the patients have acquired hypersensitivity to allergens such as food and mite antigens, which might involved in the onset of stopic diseases.
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