Vol 47, No 1 (2016)
Kazuistyka / Case reports
Published online: 2016-01-01

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Infection-related hemophagocytic lymphohistiocytosis – a case report

Karolina Torba, Grzegorz Helbig1, Krzysztof Woźniczka1, Anna Wacławik1, Sławomira Kyrcz-Krzemień1
DOI: 10.1016/j.achaem.2016.02.001
Acta Haematol Pol 2016;47(1):33-37.

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.

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