Kikuchi–fujimoto disease: features of diagnosis and clinical course

Abstract

This article describes a case of Kikuchi–Fujimoto disease (KFD) in Ukraine induced by Virus Epstein–Barr (VEB) in a one year old girl with lymphoproliferative syndrome (LPS), enteropathy, inflammatory manifestations of ENT organs, hyperthermia, leukopenia with neutropenia and thrombocytopenia, high levels of inflammatory markers, secondary hemophagocytic lymphohistiocytosis (SHLH). Empiric antibiotic therapy had no effect. Differentiation diagnosis was used to eliminate acute leukemia (AL), Myelodysplastic syndrome (MDS), infectious process and lymphoma. Bone marrow biopsy and bone marrow aspiration as well as genetic and molecular tests on bone marrow and blood were performed. The diagnosis of KFD was verified by two independent histological laboratories after histology and immunohistochemical testing on LN. A moderate amount of lymphoid cells mostly of a larger size with intensely basophilic vacuolated cytoplasm and large polymorph nucleus similar to Hodgkin cells, binucleate cell similar to Reed–Sternberg cells, macrophages, crescent shaped histiocytes were detected in the LN smears. Histology slides of LN showed a high amount of macrophages, signs of karyorrhexis and severe necrosis with absence of neutrocytes, which are common in cases of necrotizing lymphadenitis. Immunohistochemical testing showed that cells with immunoblast morphology were CD20, PAX-5 positive. Between the cells of the tumor a large amount of T-lymphocytes (CD3+) and macrophages (CD163, CD68) was detected. These cells were heterogeneously CD30-, bcl-2-positive and CD15-, CD10-, tdt-, CD38-, CD246-, bcl-6-, c-myc-negative. Expression of CD44, MUM1 та CD43 was detected in the cells of the tumor. Molecular analysis detected that most of the immunoblast cells, especially large atypical ones, were EBER EBV positive. Ki67 test showed the positivity of most of these cells and almost all of the large atypical ones. The p53 test showed the wild-type expression. The CT detected hotbeds of necrosis in a part of a lymph node and in the parenchyma of the spleen. Positive clinical effect (regression of lymphoproliferative syndrome, normalization of the hemogram, absence of laboratory sins of inflammation) was achieved after 1 month of corticosteroid treatment.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the child's parents was obtained from the studies.