open access

Vol 47, No 1 (2016)
Kazuistyka / Case reports
Published online: 2016-01-01
Submitted: 2015-12-15
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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.
Affiliations
  1. Klinika Hematologii i Transplantacji Szpiku, Śląski Uniwersytet Medyczny, Katowice, Polska

open access

Vol 47, No 1 (2016)
Kazuistyka / Case reports
Published online: 2016-01-01
Submitted: 2015-12-15

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.

Get Citation

Keywords

Hemophagocytic syndrome; Proinflammatory cytokines; Ferritin

About this article
Title

Infection-related hemophagocytic lymphohistiocytosis – a case report

Journal

Acta Haematologica Polonica

Issue

Vol 47, No 1 (2016)

Pages

33-37

Published online

2016-01-01

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

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