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Published on: March 8, 2024
Laboratory Features and Pathology of Cytokine Storm Syndromes
Flavia G Rosado1, Purva Gopal2
1Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Insights
Diagnosing cytokine storm syndromes (CSSs), like hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), is difficult. This review covers CSS pathogenesis, lab findings, and diagnostic approaches for better clinical management.
Area of Science:
- Immunology
- Hematology
- Clinical Pathology
Background:
- Cytokine storm syndromes (CSSs), including hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), pose significant diagnostic challenges in clinical practice.
- Routinely available laboratory tests for CSS diagnosis often lack specificity, complicating early and accurate identification.
- The underlying disease mechanisms of CSSs, especially in adult populations, remain incompletely understood.
Purpose of the Study:
- To review the pathogenesis of cytokine storm syndromes (CSSs).
- To detail the laboratory findings associated with CSSs.
- To outline recommended diagnostic strategies for CSSs, addressing current limitations.
Main Methods:
- Literature review focusing on pathogenesis, laboratory diagnostics, and clinical management of CSSs.
- Synthesis of information on hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS).
- Analysis of diagnostic criteria and laboratory test utility for CSS detection.
Main Results:
- Laboratory diagnosis of CSSs is challenging due to non-specific findings with standard tests.
- Confirmatory diagnostic testing for CSSs is not widely accessible in many regions, including the United States.
- Understanding of CSS pathogenesis, particularly in adults, is still evolving.
Conclusions:
- Effective laboratory diagnosis of CSSs requires a comprehensive approach, integrating clinical and evolving laboratory data.
- Improved diagnostic strategies are needed to overcome the limitations of current laboratory tests for CSSs.
- Further research into CSS pathogenesis is crucial for developing more specific diagnostic markers and targeted therapies.
Abstract:
The laboratory diagnosis of cytokine storm syndromes (CSSs), i.e., hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), is often challenging. The laboratory features using routinely available tests lack specificity, whereas confirmatory testing is available in only few laboratories in the United States. The disease mechanisms are still largely unclear, particularly in adults. In this chapter, the pathogenesis of CSSs, their associated laboratory findings, and recommended diagnostic strategies are reviewed.
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