HEMOPHAGOCYTIC SYNDROME IN A PATIENT WITH PANNICULITIS: CASE REVIEW
Summary. Hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) is a condition of the immune system hyperactivation that arises in the setting of a genetic mutation (primary HLH), infectious factors, rheumatic diseases, or malignant neoplasms. The main clinical signs of this syndrome include high fever, hepatosplenomegaly, cytopenia, hepatic cytolysis, hyperferritinemia, and coagulopathy. Aim of the study. To analyze and summarize the existing information on the diagnosis and treatment of HLH, present their own clinical experience in managing a patient. Materials and methods. The clinical case report about the development of secondary HLH was performed. Key literature data on early diagnosis and treatment of this condition were revealed. Results. This article describes a clinical case of rapid deterioration with the development of multiorgan failure on the background of hemophagocytic syndrome in a 39-years old woman with initially diagnosed Weber-Christian panniculitis. The key points of differential diagnosis symptoms of the underlying disease and hemophagocytic syndrome, determination of specific laboratory markers (especially sCD25/sIL-2R), treatment with glucocorticoids, and etoposide are disclosed. Conclusions. The presence of certain common features of HLH and the underlying rheumatic disease may complicate the differential diagnosis and delay the start of treatment. Early recognition of HLH symptoms simultaneously with the beginning of adequate therapy can improve the prognosis of patient survival.
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