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Infection-related hemophagocytic lymphohistiocytosis – a case report
Affiliations- Klinika Hematologii i Transplantacji Szpiku, Śląski Uniwersytet Medyczny, Katowice, Polska
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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.
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.
Keywords
Hemophagocytic syndrome; Proinflammatory cytokines; Ferritin
About this articleTitle
Infection-related hemophagocytic lymphohistiocytosis – a case report
Journal
Issue
Pages
33-37
Published online
2016-01-01
Page views
103
Article views/downloads
276
DOI
10.1016/j.achaem.2016.02.001
Bibliographic record
Acta Haematol Pol 2016;47(1):33-37.
Keywords
Hemophagocytic syndrome
Proinflammatory cytokines
Ferritin
Authors
Karolina Torba
Grzegorz Helbig
Krzysztof Woźniczka
Anna Wacławik
Sławomira Kyrcz-Krzemień