Hlh up to date8/2/2023 Ī unique flow cytometric (FCM) finding in Epstein-Barr virus (EBV)-positive HLH patients has been reported: clonal expansion of EBV-infected CD8 + T cells with CD5 or CD7 downregulation. Morphological evaluation of BM aspirates in HLH patients usually shows non-specific abnormal findings such as an increased number of histiocytes and lymphocytes with or without hemophagocytosis and a marked left shift in myelopoiesis. ![]() ![]() Because these clinical and laboratory findings are often present in various diseases, the diagnosis of HLH may be challenging, especially in early stages of disease. It is characterized by fever, splenomegaly, cytopenia, hypertriglyceridaemia, hypofibrinogenemia, low or absent natural killer (NK) cell activity, elevated blood levels of the soluble IL-2 receptor and ferritin, and haemophagocytosis in biopsy samples of bone marrow (BM), spleen or lymph nodes. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome related to excessive immune activation of macrophages and T cells. Keywords: Epstein-Barr virus, Familial, Hemophagocytic lymphohistiocytosis, Flow cytometry, T cell, Overall survival However, it may not be useful as a prognostic marker. ConclusionsĪn aberrant T cell immunophenotype may assist in discriminating EBV-negative secondary HLH and EBV-positive HLH. The presence of an aberrant T cell immunophenotype was not related to overall survival in EBV-positive HLH and EBV-negative secondary HLH patients. Only one (1/15, 6.7%) patient with EBV-negative secondary HLH showed an aberrant loss of CD7 antigen on CD8 + T cells. ResultsĪn aberrant immunophenotype, including CD5 and/or CD7 downregulation on T cells, was observed in 55.6% (15/27) of the EBV-positive HLH patients and 100% of the familial HLH (3/3). Overall survival was estimated using Kaplan-Meier curves and compared using the log-rank test. We retrospectively characterized downregulation patterns of CD5 or CD7 on activated T cells, using flow cytometry. We classified 45 HLH patients into three subgroups: EBV-positive HLH (N=27), EBV-negative secondary HLH (N=15), and familial HLH (N=3). As the utility of T cell immunophenotypes as an adjunctive diagnostic or a prognostic marker for HLH has not been evaluated, we analyzed T cell immunophenotypes in HLH patients for this purpose. Downregulation of CD5 or CD7 on T cells has been reported in patients with Epstein-Barr virus (EBV)-positive HLH. T cell immunophenotypes in patients with hemophagocytic lymphohistiocytosis (HLH) have been described.
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