open access

Ahead of print
Review article
Submitted: 2021-11-01
Accepted: 2021-11-10
Published online: 2021-11-24
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Hemophagocytic lymphohistiocytosis — what's new in old diagnostic and clinical criteria?

Małgorzata Salamonowicz-Bodzioch1
DOI: 10.5603/AHP.a2021.0102
Affiliations
  1. Department of Children Oncology and Hematology, Wroclaw Medical University, Wrocław, Poland

open access

Ahead of print
REVIEW ARTICLE
Submitted: 2021-11-01
Accepted: 2021-11-10
Published online: 2021-11-24

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a condition of overexpressed inflammatory response resulting in hypercytokinemia, macrophages infiltration and subsequent multiple organ failure. Without treatment, it leads to death. The main etiological factors include : viral, bacterial and parasitic infections, malignancies and autoinflammatory diseases . The main clinical manifestations are: high fever ≥ 38 °C , lymphadenopathy, splenomegaly, hepatomegaly. Central nervous system involvement occurs in 30-70% of cases. Less common symptoms include: dyspnea, cough, arrhythmias, jaundice, peripheral edema, rashes, albinism and diarrhea. The picture of the disease seen in laboratory tests consists of : duopenia, hypofibrinogenemia ( < 150 mg/dl) high D-dimers level, hyperferritinemia . Other abnormalities include hypertiglyceridemia , elevated liver enzymes, hyperbilirubinemia, hypoalbuminemia and hyponatremia. Diagnostics include : laboratory tests , histopathological examination, lumbar puncture, radiological imaging, functional test and genetic checking. It is important to rule out factors mimicking HLH. Some of the old, well known criteria are no more relevant nowadays. The aim of the therapy is immunosuppressive, immunomodulatory and anti-cytokine treatment, using HLH-2004 protocol. In secondary HLH, elimination of the causative agent is critical. In case of primary HLH, or relapse of secondary forms allogenic transplantation is the only curative treatment. The prognosis is uncertain.

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a condition of overexpressed inflammatory response resulting in hypercytokinemia, macrophages infiltration and subsequent multiple organ failure. Without treatment, it leads to death. The main etiological factors include : viral, bacterial and parasitic infections, malignancies and autoinflammatory diseases . The main clinical manifestations are: high fever ≥ 38 °C , lymphadenopathy, splenomegaly, hepatomegaly. Central nervous system involvement occurs in 30-70% of cases. Less common symptoms include: dyspnea, cough, arrhythmias, jaundice, peripheral edema, rashes, albinism and diarrhea. The picture of the disease seen in laboratory tests consists of : duopenia, hypofibrinogenemia ( < 150 mg/dl) high D-dimers level, hyperferritinemia . Other abnormalities include hypertiglyceridemia , elevated liver enzymes, hyperbilirubinemia, hypoalbuminemia and hyponatremia. Diagnostics include : laboratory tests , histopathological examination, lumbar puncture, radiological imaging, functional test and genetic checking. It is important to rule out factors mimicking HLH. Some of the old, well known criteria are no more relevant nowadays. The aim of the therapy is immunosuppressive, immunomodulatory and anti-cytokine treatment, using HLH-2004 protocol. In secondary HLH, elimination of the causative agent is critical. In case of primary HLH, or relapse of secondary forms allogenic transplantation is the only curative treatment. The prognosis is uncertain.
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Keywords

Hemophagocytic syndrome; hypercytokinemia; Macrophage activation syndrome

About this article
Title

Hemophagocytic lymphohistiocytosis — what's new in old diagnostic and clinical criteria?

Journal

Acta Haematologica Polonica

Issue

Ahead of print

Article type

Review article

Published online

2021-11-24

DOI

10.5603/AHP.a2021.0102

Keywords

Hemophagocytic syndrome
hypercytokinemia
Macrophage activation syndrome

Authors

Małgorzata Salamonowicz-Bodzioch

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