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Posttransplant lymphoproliferative disorder (PTLD) is a lymphoid and/or plasma cell proliferation that occurs as a result of immunosuppressive therapy prescribed to patients to prevent rejection of the transplanted organ, in the context of solid organ or allogeneic hematopoietic cell transplantation. The trigger for the development of PTLD can be active replication of Epstein-Barr virus (EBV), as well as other risk factors. This disorder represents a spectrum of clinical conditions, from benign disease similar to mononucleosis to fulminant lymphoma. Early recognition of PTLD is important in organ transplant patients because it tends to progress rapidly. Familiarity with the clinical features of PTLD and increased vigilance are essential to establishing the diagnosis. Aim - to describe a clinical case of PTLD associated with EBV replication in a 7-year-old girl with congenital podocytopathy associated with WT1 gene mutation after kidney transplantation from a related donor. The importance of timely diagnosis and treatment of this disorder to improve prognosis and reduce risks in patients with similar clinical characteristics is emphasized. Clinical case. The case of a 7-year-old girl with congenital podocytopathy associated with the WT1 gene mutation after a family kidney transplant is described. The child was diagnosed with polylymphadenopathy and febrile fever. An increase in acute-phase parameters and deterioration of the transplanted organ function were observed. The results of computed tomography showed polylymphadenopathy, subpleural, peribronchovascular foci in the lung parenchyma. For the diagnosis of PTLD, lymph node biopsy and determination of the number of EBV copies by polymerase chain reaction were performed. Differential diagnosis with lymphoma was performed. Pathologic examination of the nodes confirmed PTLD associated with EBV. Improvement was achieved after the use of targeted therapy with anti-CD20 monoclonal antibodies (rituximab, 4 injections). The article emphasizes the importance of timely diagnosis of PTLD, which is a serious complication that can occur after transplantation. The dynamics of EBV detection is important in high-risk situations. Immunotherapy with monoclonal anti-B-cell antibodies (anti-CD20) plays a key role in the treatment of PTLD. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.
Дорош et al. (Tue,) studied this question.