Clinical Characteristics of Adult Hemophagocytic Lymphohistiocytosis in the Emergency Department

Abstract

Purpose

To determine the clinical manifestations and results of adult hemophagocytic lymphohistiocytosis (HLH) patients in our emergency department.

Methods

We retrospectively evaluated patients with HLH from 1 April 2018 to 31 December 2020. The clinical data of these patients (basic information, symptoms, vital signs, laboratory results, HLH diagnostic criteria, H Score, main treatments, outcomes) were collected.

Results

Thirty-three patients (23 males and 10 females; 40.55±18.78 years) with 34 clinical episodes (one male had two clinical episodes and died during the second episode) were enrolled. Twenty-five patients were placed in a “survivor” group, and nine patients were categorized into a “deceased” group. Fever, splenomegaly, hemoglobin <90 g/L and platelet count <100×10⁹/L most commonly met the diagnostic standard for HLH. The H Score results in the survival group and deceased group was 212.4±37.18 and 252.1±40.95, respectively. Viral infection was the most common reason for HLH, followed by immune-system disease and cancer. Laboratory tests showed that deceased-group patients had multiple-organ dysfunction. Multivariate logistic regression showed that the lactate dehydrogenase (lactate dehydrogenase) level (P = 0.039; odds ratio, 0.999) was significantly related to death.

Conclusion

In the emergency department, HLH should be considered for critically ill patients with fever, splenomegaly, low hemoglobin and low platelet count. The H Score might be useful to diagnose HLH quickly. In our study, 26.47% of HLH patients died in the emergency department, and patients with a significantly increased lactate dehydrogenase level had a markedly increased risk of death.

Keywords:

• clinical characteristics
• hemophagocytic lymphohistiocytosis
• emergency department
• mortality
• lactate dehydrogenase

Data Sharing Statement

All data generated or analyzed during this study are included in the manuscript.

Consent for Publication

All authors have approved the manuscript for submission.

Disclosure

The authors declare that there are no conflicts of interest.

Additional information

Funding

This work was supported by the National Natural Science Foundation of China (81501923) and the Rui E (Ruiyi) Emergency Medical Research Special Funding Project (R2019007).