Limfohistiocytoza hemofagocytarna zwi ązana z infekcją – opis przypadku

Publication date: January–March 2016 Source:Acta Haematologica Polonica, Volume 47, Issue 1 Author(s): Karolina Torba, Grzegorz Helbig, Krzysztof Woźniczka, Anna Wacławik, Sławomira Kyrcz-Krzemień 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.

Source: Acta Haematologica Polonica - July 20, 2016 Category: Hematology Source Type: research