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Chapter 27 - Hemophagocytic Lymphohistiocytosis (HLH)

from Section VI - Histiocytic Disorders and Neoplasms

Published online by Cambridge University Press:  25 January 2024

By
Jennifer Picarsic  and
Rebecca Marsh
Edited by
Xiayuan Liang ,
Bradford Siegele  and
Jennifer Picarsic
Xiayuan Liang
Affiliation:
Children’s Hospital of Colorado
Bradford Siegele
Affiliation:
Children’s Hospital of Colorado
Jennifer Picarsic
Affiliation:
Cincinnati Childrens Hospital Medicine Center
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Summary

Hemophagocytic lymphohistiocytosis (HLH) is a complex, life-threatening clinical syndrome of systemic hyperinflammation. The syndrome is often diagnosed based on the presence of clinical signs and symptoms that were included as diagnostic criteria for HLH by the Histiocyte Society in clinical treatment trials (1, 2). The criteria include fever, splenomegaly, cytopenias, hypertriglyceridemia and/or hypofibrinogenemia, observation of hemophagocytosis, decreased function of natural killer (NK) cells, elevated ferritin, and elevated soluble interleukin-2 (IL-2) receptor levels (Table 27.1). Other complications of HLH that are not part of the criteria include central nervous system (CNS) involvement in 30–70% of patients (3, 4), as well as hepatitis or acute liver failure. Rarely, isolated CNS disease can also occur (5). The syndrome of HLH can be caused by a wide variety of etiologies, and it is imperative that clinicians bear this in mind when the clinical diagnosis is suspected (6).

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Chapter
Information
Pediatric Pathology of Hematopoietic and Histiocytic Disorders , pp. 320 - 329
Publisher: Cambridge University Press
Print publication year: 2024

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References

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