Elsevier

Acta Haematologica Polonica

Volume 47, Issue 1, January–March 2016, Pages 33-37
Acta Haematologica Polonica

Kazuistyka/Case report
Limfohistiocytoza hemofagocytarna związana z infekcją – opis przypadkuInfection-related hemophagocytic lymphohistiocytosis – a case report

https://doi.org/10.1016/j.achaem.2016.02.001Get rights and content

Abstract

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.

Słowa kluczowe

zespół hemofagocytarny
cytokiny prozapalne
ferrytyna

Keywords

Hemophagocytic syndrome
Proinflammatory cytokines
Ferritin

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