Limfohistiocytoza hemofagocytarna związana z infekcją - opis przypadku

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• 作者：Karolina Torba ; ^{karolina.torbaa@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Grzegorz Helbig ; Krzysztof Woźniczka ; Anna Wacławik ; Sławomira Kyrcz-Krzemień
• 关键词：zespó ; ł hemofagocytarny ; cytokiny prozapalne ; ferrytyna
• 刊名：Acta Haematologica Polonica
• 出版年：2016
• 出版时间：January-March 2016
• 年：2016
• 卷：47
• 期：1
• 页码：33-37
• 全文大小：828 K

文摘

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disorder characterized by abnormal activation of macrophages. It is also characterized by hemophagocytosis in the bone marrow and in the reticuloendothelial system (RES). The most common symptoms are persistent fever, splenomegaly and cytopenia. The probable mechanism of disease is due to hyperinflammation caused by increasing amounts of proinflammatory cytokines. As a consequence numerous metabolic disturbances with multiple organ failure occur. Without a proper treatment this disease may have a fatal outcome. Herein we present a 24-year-old male with HLH who achieved a rapid response to the therapy despite the initial poor overall condition which was associated with an advanced disease stage as well as prolonged diagnostic process.